Altered Thymidine Metabolism Due to Defects of Thymidine Phosphorylase

Name: Work Video:
Duration: 3 min 30 s
Antonella Spinazzola; Ramón Martí; Ichizo Nishino; Antoni L. Andreu; Ali Naini; Saba Tadesse; Ivana Pela; Enrico Zammarchi; Maria Alice Donati; Juan Oliver; Michio Hirano
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'is_corresponding': False, 'raw_author_name': 'Ichizo Nishino', 'raw_affiliation_strings': ['Dept. of Neurology, Columbia University College of Physicians and Surgeons, P&S 4-443, 630 West 168th St., New York, NY 10032.', 'From the ‡Department of Neurology, Columbia University College of Physicians and Surgeons, New York, New York 10032, the ¶Department of Pediatrics,', "University of Florence, Meyer Children's Hospital, 50132 Florence, Italy,", 'ʈDepartment of Medicine, Columbia University College of Physicians and Surgeons, New York, New York 10032'], 'affiliations': [{'raw_affiliation_string': 'Dept. of Neurology, Columbia University College of Physicians and Surgeons, P&S 4-443, 630 West 168th St., New York, NY 10032.', 'institution_ids': ['https://openalex.org/I78577930']}, {'raw_affiliation_string': 'From the ‡Department of Neurology, Columbia University College of Physicians and Surgeons, New York, New York 10032, the ¶Department of Pediatrics,', 'institution_ids': 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Andreu', 'orcid': 'https://orcid.org/0000-0002-3936-3234'}, 'institutions': [{'id': 'https://openalex.org/I78577930', 'display_name': 'Columbia University', 'ror': 'https://ror.org/00hj8s172', 'country_code': 'US', 'type': 'education', 'lineage': ['https://openalex.org/I78577930']}, {'id': 'https://openalex.org/I4210113625', 'display_name': "Meyer Children's Hospital", 'ror': 'https://ror.org/01n2xwm51', 'country_code': 'IT', 'type': 'healthcare', 'lineage': ['https://openalex.org/I4210113625']}, {'id': 'https://openalex.org/I45084792', 'display_name': 'University of Florence', 'ror': 'https://ror.org/04jr1s763', 'country_code': 'IT', 'type': 'education', 'lineage': ['https://openalex.org/I45084792']}], 'countries': ['IT', 'US'], 'is_corresponding': False, 'raw_author_name': 'Antonio L. Andreu', 'raw_affiliation_strings': ['Dept. of Neurology, Columbia University College of Physicians and Surgeons, P&S 4-443, 630 West 168th St., New York, NY 10032.', 'From the ‡Department of Neurology, Columbia University College of Physicians and Surgeons, New York, New York 10032, the ¶Department of Pediatrics,', "University of Florence, Meyer Children's Hospital, 50132 Florence, Italy,", 'ʈDepartment of Medicine, Columbia University College of Physicians and Surgeons, New York, New York 10032'], 'affiliations': [{'raw_affiliation_string': 'From the ‡Department of Neurology, Columbia University College of Physicians and Surgeons, New York, New York 10032, the ¶Department of Pediatrics,', 'institution_ids': ['https://openalex.org/I78577930']}, {'raw_affiliation_string': "University of Florence, Meyer Children's Hospital, 50132 Florence, Italy,", 'institution_ids': ['https://openalex.org/I4210113625', 'https://openalex.org/I45084792']}, {'raw_affiliation_string': 'ʈDepartment of 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Zammarchi', 'raw_affiliation_strings': ['Dept. of Neurology, Columbia University College of Physicians and Surgeons, P&S 4-443, 630 West 168th St., New York, NY 10032.', 'From the ‡Department of Neurology, Columbia University College of Physicians and Surgeons, New York, New York 10032, the ¶Department of Pediatrics,', "University of Florence, Meyer Children's Hospital, 50132 Florence, Italy,", 'ʈDepartment of Medicine, Columbia University College of Physicians and Surgeons, New York, New York 10032'], 'affiliations': [{'raw_affiliation_string': 'From the ‡Department of Neurology, Columbia University College of Physicians and Surgeons, New York, New York 10032, the ¶Department of Pediatrics,', 'institution_ids': ['https://openalex.org/I78577930']}, {'raw_affiliation_string': "University of Florence, Meyer Children's Hospital, 50132 Florence, Italy,", 'institution_ids': ['https://openalex.org/I4210113625', 'https://openalex.org/I45084792']}, {'raw_affiliation_string': 'ʈDepartment of 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2695, 2699, 2716, 2726, 2739, 2749, 2759, 2866, 2942, 2949, 2954, 3001, 3038, 3052, 3056, 3099, 3115, 3131, 3136, 3150, 3176, 3188, 3200, 3210, 3232, 3251, 3256, 3271, 3321, 3423, 3440, 3504, 3515, 3525, 3709, 3715, 3743, 3758, 3763, 3781, 3892, 3903, 3935, 3940, 3975, 3977, 3986, 4005, 4016, 4048, 4059, 4063, 4069, 4160, 4201, 4204, 4222, 4250, 4260, 4262, 4287, 4302, 4322], 'thymine': [26, 216, 902, 1234, 1421, 1439, 1451, 2552], 'and': [27, 70, 88, 152, 167, 217, 260, 278, 342, 357, 398, 447, 469, 482, 596, 685, 703, 705, 781, 808, 903, 953, 960, 980, 1019, 1044, 1159, 1180, 1193, 1200, 1313, 1316, 1334, 1381, 1417, 1438, 1474, 1490, 1493, 1516, 1522, 1544, 1556, 1647, 1691, 1736, 1773, 1776, 1783, 1844, 1909, 1933, 1948, 1958, 1999, 2052, 2064, 2072, 2194, 2212, 2297, 2345, 2453, 2553, 2713, 2823, 2878, 3006, 3017, 3061, 3134, 3193, 3377, 3446, 3466, 3496, 3507, 3559, 3615, 3670, 3920, 3925, 4025, 4066, 4282], '2-deoxy-d-ribose': [28, 218, 904, 2554], '1-phosphate.': [29, 219, 2555], 'We': [30, 159, 220, 349, 825, 948, 971, 1205, 1671, 1694, 1745, 1755, 1858, 2027, 2277, 2752, 2862, 2992, 3457, 3911, 4293], 'present': [31, 221, 2984, 3266], 'evidence': [32, 222, 1022, 2953], 'that': [33, 85, 92, 161, 223, 275, 282, 351, 465, 2036, 2107, 2234, 2330, 2631, 2765, 2900, 2981, 3079, 3121, 3129, 3250, 3254, 3438, 3490, 3512, 3552, 3633, 3659, 3726, 3869, 3913, 4012, 4026], 'metabolism': [35, 225, 2957, 3478], 'altered': [37, 227, 2955, 3908, 3919], 'MNGIE.': [39, 229, 2500, 3931], 'activities': [41, 231, 1870, 1898, 2614, 2738], 'buffy': [43, 150, 233, 340, 1872, 3444, 4280], 'coats': [44, 151, 234, 341, 1294, 1873, 3445, 4281], 'were': [45, 235, 1145, 1173, 1295, 1322, 1337, 1461, 1488, 1503, 1511, 1526, 1530, 1547, 1568, 1598, 1840, 1874, 1902, 1911, 1963, 2038, 2105, 2446, 3203], 'reduced': [46, 236, 1876, 1913, 2181, 2260, 2604, 2737, 3215], 'drastically': [47, 237, 2603], 'all': [49, 239, 1041, 1803, 1878, 1900, 2271], '27': [50, 240, 973, 1042, 1879], 'MNGIE': [51, 58, 120, 188, 241, 248, 310, 378, 680, 842, 921, 958, 988, 1149, 1689, 1969, 2268, 2311, 2354, 2426, 2606, 2707, 2959, 2975, 3002, 3087, 3123, 3157, 3183, 3403, 3455, 3491, 3522, 3948, 3980, 4039, 4223, 4265, 4297], 'patients': [52, 59, 138, 242, 249, 328, 959, 966, 1128, 1150, 1690, 1880, 1901, 1932, 1970, 2071, 2093, 2272, 2296, 2312, 2607, 2708, 3088, 3100, 3184, 3404, 3475, 3492, 3929, 3981, 4238, 4266, 4324], 'compared': [53, 243, 1885, 1920, 1979, 2315, 2608, 2709, 3206, 3248], 'with': [54, 244, 593, 689, 784, 797, 819, 975, 1129, 1140, 1374, 1549, 1600, 1657, 1711, 1833, 1886, 1921, 1980, 2022, 2042, 2316, 2329, 2531, 2609, 2628, 2710, 2776, 2859, 2874, 3113, 3143, 3207, 3249, 3500, 3930], '19': [55, 245, 1196, 1887], 'controls.': [56, 246, 983, 1693], 'All': [57, 247], 'had': [60, 250, 2247], 'much': [61, 251], 'higher': [62, 252, 2778, 2905, 3433], 'plasma': [63, 135, 156, 253, 325, 346, 954, 1491, 1523, 1967, 2020, 2063, 2208, 2222, 2758, 4285], 'levels': [64, 154, 183, 254, 344, 373, 956, 1525, 1962, 1993, 2243, 2748, 3008, 3063, 3135, 3231, 3356, 3514, 4301], 'than': [67, 257, 473, 486, 2704, 2770, 3541, 3735], 'normal': [68, 258, 982, 1856, 1906, 1981, 2095, 2622, 2711, 2760, 2830], 'individuals': [69, 259], 'asymptomatic': [71, 261, 978, 2714], 'mutation': [73, 263, 1986], 'carriers.': [74, 264], 'In': [75, 109, 124, 137, 265, 299, 314, 327, 920, 1498, 2946, 3521, 4255], 'two': [76, 266, 444, 965, 1464, 1499, 1767, 2070, 2090, 2187, 2310, 3096, 3181], 'patients,': [77, 267, 922, 1500, 3220, 3523, 4298], 'renal': [79, 269, 2096, 2119, 3198, 3245, 3319, 3346, 3414, 4202], 'clearance': [80, 270, 3209, 3320, 3415], 'was': [83, 273, 1214, 1235, 1346, 1372, 1401, 1415, 1423, 1445, 1478, 1623, 1631, 1642, 1831, 1935, 1942, 2010, 2076, 2086, 2230, 2327, 2337, 3032, 3214, 3328, 3350], '∼20%': [84, 274, 2106], 'creatinine,': [87, 277, 2099, 3252], 'because': [89, 279, 2735, 3042, 3167, 3972], 'hemodialysis': [90, 280, 1169, 3217, 4024], 'demonstrated': [91, 281, 3480, 3706], 'ultrafiltratable,': [95, 285, 3242], 'most': [96, 286, 2682, 3255], 'filtered': [99, 289, 3258], 'likely': [102, 292, 3275, 3897], 'be': [104, 185, 294, 375, 452, 583, 2577, 2588, 2692, 3482, 3754, 3950, 3967, 4032, 4271, 4326], 'reabsorbed': [105, 295], 'by': [106, 118, 296, 308, 454, 1220, 1403, 1480, 1648, 1824, 2012, 2050, 2266, 2384, 2870, 3216, 3484, 3645, 3655, 3756, 4051, 4206], 'kidney.': [108, 298], 'vitro,': [110, 300], 'fibroblasts': [111, 121, 301, 311, 1686, 2258, 2269, 2294, 2305, 2322, 2396, 2950, 2962, 2976, 3003], 'from': [112, 302, 957, 1126, 1463, 1575, 1592, 1687, 1697, 1718, 1743, 1802, 1810, 1968, 2270, 2295, 2309, 2323, 2819, 2884, 3070, 3076, 3086, 3960, 3993, 4274], 'controls': [113, 303, 1888, 1925, 1982, 2298, 2333, 2712, 2761, 3160], 'catabolized': [114, 304], 'medium;': [117, 307, 2265], 'contrast,': [119, 309, 565, 2267, 2971, 3180, 3340], 'released': [122, 312, 2274, 4020], 'thymidine.': [123, 136, 313, 326, 1670, 2026, 2696, 2751, 2991, 4060], 'MNGIE,': [125, 144, 315, 334, 976], 'severe': [126, 316, 927, 998], 'impairment': [127, 317], 'activity': [131, 148, 321, 338, 932, 952, 1444, 1957, 2601, 2689, 2728, 3442, 3495, 3891, 4277], 'leads': [132, 322], 'increased': [134, 324, 1965, 3406], 'who': [139, 329, 1988], 'are': [140, 157, 330, 347, 440, 579, 2413, 2482, 2618, 2701, 2743, 2974, 3449, 3458, 3554, 3918, 3937, 4018, 4286], 'suspected': [141, 331], 'having': [143, 333], 'determination': [145, 335, 3439], 'diagnostic.': [158, 348], 'hypothesize': [160, 350, 3912, 4294], 'excess': [162, 352, 1660], 'alters': [164, 354], 'mitochondrial': [165, 173, 355, 363, 380, 409, 449, 798, 1024, 3508, 3518, 3634, 3711, 3750, 3765, 3783, 3854, 3904, 3915, 3941], 'nucleoside': [166, 356, 2226, 2246, 3202, 3234, 3259, 3418, 3712, 4050, 4070, 4209], 'nucleotide': [168, 358, 2540, 4305], 'pools': [169, 359, 3536, 3563, 3714, 3752, 3906, 3917, 4306], 'leading': [170, 360, 4309], 'impaired': [172, 362], 'DNA': [174, 364, 3519, 3635, 3942], 'replication,': [175, 365], 'repair,': [176, 366], 'or': [177, 367, 459, 576, 692, 788, 816, 1006, 1032, 1700, 1865, 1954, 1983, 2723, 2762, 3171, 3647, 3666], 'both.': [178, 368, 789], 'Therapies': [179, 369], 'reduce': [181, 371, 3952, 4317], 'may': [184, 374, 2576, 3982, 4030, 4325], 'beneficial': [186, 376], 'patients.': [189, 379, 2091, 2217, 2960, 3158, 3456, 4040, 4224], 'neurogastrointestinal': [381], 'encephalomyopathy': [382], 'triphosphate': [386, 388, 2592, 3534, 3713], 'deoxynucleoside': [387, 3533], 'high': [389, 2087, 3186, 3345, 3513, 3933], 'pressure': [390], 'liquid': [391], 'chromatography': [392], 'Mitochondrial': [393], 'encephalomyopathies': [394, 450], 'encompass': [395], 'a': [396, 474, 513, 1156, 1217, 1385, 1611, 1651, 1658, 1818, 2043, 2153, 2360, 2419, 2456, 2529, 2634, 2931, 2996, 3023, 3092, 3109, 3302, 3764, 3968, 4214, 4227, 4258], 'diverse': [397], 'expanding': [399], 'group': [400, 2370, 3067], 'disorders': [403], 'defects': [406, 455, 570], 'respiratory': [410, 525, 821], 'chain': [411, 439, 526, 822], '(1DiMauro': [412], 'S.': [413, 499, 555, 632, 661, 714, 742, 754, 852, 1057, 1085, 1097, 1263, 1278, 3842, 3878], 'Bonilla': [414, 613, 749, 1092], 'E.': [415, 539, 614, 750, 1093, 3309, 4182], 'Davidson': [416], 'M.': [417, 419, 535, 537, 604, 649, 655, 756, 844, 887, 938, 1099, 1115, 1245, 3821], 'Hirano': [418, 747, 755, 886, 937, 1090, 1098, 1114], 'Schon': [420], 'E.A.': [421], 'Biochim.': [422, 2642, 3290, 4088, 4103], 'Biophys.': [423, 2643, 3291, 4089, 4104], 'Acta.': [424, 2644, 3292, 4090, 4105], '1998;': [425, 671, 869, 912, 2519], '1366:': [426], '199-210Crossref': [427], 'PubMed': [428, 508, 549, 561, 637, 674, 762, 877, 892, 915, 943, 1105, 1120, 1257, 1270, 1290, 1360, 2522, 2648, 2661, 2793, 2813, 2847, 2925, 3296, 3577, 3595, 3611, 3685, 3703, 3801, 3817, 3848, 3884, 4094, 4109, 4133, 4151, 4174, 4193], 'Scopus': [429, 550, 675, 763, 878, 893, 916, 944, 1106, 1121, 1271, 1361, 2523, 2649, 2662, 2794, 2814, 2848, 2926, 3297, 3835, 3849, 3885, 4095, 4110, 4134, 4175, 4194], '(140)': [430], 'Google': [431, 509, 552, 562, 638, 677, 765, 880, 895, 918, 946, 1108, 1123, 1258, 1273, 1291, 1363, 2525, 2651, 2664, 2796, 2816, 2850, 2928, 3299, 3315, 3375, 3401, 3578, 3596, 3612, 3630, 3686, 3704, 3802, 3818, 3837, 3851, 3887, 4097, 4112, 4136, 4152, 4177, 4196], 'Scholar).': [432, 510, 563, 678, 766, 896, 919, 947, 1124, 1292, 1364, 2526, 3300, 3402, 3613, 3852, 3888, 4153, 4197], 'Because': [433, 3239, 3742], 'subunits': [434], 'electron': [437], 'transport': [438], 'products': [442], 'genomes,': [445], 'nDNA': [446, 517, 568], 'mtDNA,': [448, 1144], 'can': [451, 4156], 'caused': [453], 'either': [457, 813], 'genome': [458], 'cross-talk': [461], 'between': [462, 682, 1436, 1853, 1931, 1945, 1955, 2340, 2364, 2998, 3058], 'genomes': [464], 'regulate': [466], 'mtDNA': [467, 481, 574, 599, 3505, 3923, 4311], 'integrity': [468], 'quantity.': [470], 'For': [471, 1167], 'more': [472, 485, 2703, 3080, 3083, 3537, 3729], 'decade,': [475], 'investigators': [476], 'have': [477, 483, 519, 3169, 3343, 3405, 3557, 4075], 'focused': [478], 'primarily': [479], 'upon': [480, 1644], 'identified': [484, 521, 826, 1030], '100': [487, 1728], 'distinct': [488], 'point': [489], 'as': [491, 493, 522, 701, 801, 838, 995, 1447, 1807, 1809, 1817, 2032, 2054, 2666, 2898, 3247, 4239, 4241, 4257], 'well': [492, 1808, 2509, 3127], 'large': [494, 1659, 4013], 'scale': [495], 'molecular': [496], 'rearrangement': [497], '(2Servidei': [498], 'Neuromuscul.': [500], 'Disord.': [501], '2001;': [502], '11:': [503, 547], '230-235Abstract': [504], 'Full': [505, 872, 874, 1254, 1287, 2810, 2922, 3574, 3592, 3608, 3627, 3682, 3700, 3798, 3814, 3830, 3832, 4128, 4130], 'Text': [506, 873, 875, 1255, 1288, 2811, 2923, 3575, 3593, 3609, 3628, 3683, 3701, 3799, 3815, 3831, 3833, 4129, 4131], 'PDF': [507, 876, 1256, 1289, 2812, 2924, 3576, 3594, 3610, 3629, 3684, 3702, 3800, 3816, 3834, 4132], 'Since': [511], '1995,': [512], 'growing': [514, 3094, 3110], 'number': [515, 1787, 2341, 2437, 3000, 3016, 3060], 'been': [520, 2508, 4076], 'causes': [523, 3517], 'monoenzymopathies': [527], '(3Bourgeron': [528], 'T.': [529, 3385], 'Rustin': [530], 'P.': [531], 'Chretien': [532], 'D.': [533, 1247, 3367, 3618], 'Birch-Machin': [534], 'Bourgeois': [536], 'Viegas-Péquignot': [538], 'Munnich': [540], 'A.': [541, 543, 610, 641, 647, 653, 710, 712, 722, 885, 936, 1053, 1055, 1065, 1113, 3823, 4117], 'Rötig': [542], 'Nat.': [544], 'Genet.': [545, 868], '1995;': [546, 3845, 3881], '144-149Crossref': [548], '(622)': [551], 'Scholar,': [553, 639, 881, 1109, 1259, 1274, 2652, 2797, 3579, 3597, 3687, 3803, 3819, 3838, 4098, 4113, 4137, 4178], '4DiMauro': [554], 'Ann.': [556, 757, 1100], 'Neurol.': [557, 758, 1101], '1999;': [558, 889, 940, 1117], '45:': [559], '693-694Crossref': [560], 'By': [564, 2970, 3179, 3339], 'causing': [569], 'intergenomic': [572], 'signals,': [573], 'depletion': [575, 597, 3924], 'multiple': [577, 594, 820, 1141, 3926, 4027], 'deletions,': [578], 'just': [580], 'beginning': [581], 'defined.': [584], '(MNGIE)1': [586], 'associated': [592, 1139], 'deletions': [595, 1142, 3927], 'skeletal': [601, 694], 'muscle': [602, 791], '(5Hirano': [603], 'Silvestri': [605], 'G.': [606, 657, 665, 669], 'Blake': [607], 'D.M.': [608], 'Lombes': [609], 'Minetti': [611], 'C.': [612], 'Hays': [615], 'A.P.': [616], 'Lovelace': [617], 'R.E.': [618], 'Butler': [619], 'I.': [620, 663, 708, 716, 726, 746, 748, 850, 883, 934, 1051, 1059, 1069, 1089, 1091, 1111], 'Bertorini': [621], 'T.E.': [622], 'Threlkeld': [623], 'A.B.': [624], 'Mitsumoto': [625], 'H.': [626, 728, 1071], 'Salberg': [627, 859], 'L.': [628, 651, 2799, 2911], 'Rowland': [629, 751, 1094], 'L.P.': [630, 752, 1095], 'DiMauro': [631, 753, 851, 1096], 'Neurology.': [633, 670], '1994;': [634], '44:': [635], '721-727Crossref': [636], '6Papadimitriou': [640], 'Comi': [642], 'G.P.': [643], 'Hadjigeorgiou': [644, 737, 1080], 'G.M.': [645, 738, 1081], 'Bordoni': [646], 'Sciacco': [648], 'Napoli': [650], 'Prelle': [652], 'Moggio': [654], 'Fagiolari': [656], 'Bresolin': [658], 'N.': [659], 'Salani': [660], 'Anastasopoulos': [662], 'Giassakis': [664], 'Divari': [666], 'R.': [667, 909, 2516, 4142], 'Scarlato': [668], '51:': [672], '1086-1092Crossref': [673], '(96)': [676], 'Clinically,': [679], 'presents': [681], '1st': [684], '5th': [687], 'decades': [688], 'ptosis,': [690, 1004], 'ophthalmoparesis,': [691, 1005], 'both;': [693, 1007], 'myopathy;': [695], 'peripheral': [696, 782, 1009], 'neuropathy;': [697, 1010], 'gastrointestinal': [698, 999], 'dysmotility,': [699], 'manifesting': [700], 'diarrhea': [702], 'pseudo-obstruction;': [704], 'cachexia': [706], '(7Nishino': [707, 1050], 'Spinazzola': [709, 884, 935, 1052, 1112], 'Papadimitriou': [711, 1054], 'Hammans': [713, 1056], 'Steiner': [715, 1058], 'Hahn': [717, 857, 1060], 'C.D.': [718, 1061], 'Connolly': [719, 1062], 'A.M.': [720, 1063], 'Verloes': [721, 1064], 'Guimarães': [723, 1066], 'J.': [724, 846, 866, 911, 1067, 1248, 1264, 1281, 2518, 2637, 2805, 2917, 3568, 3586, 3602, 3621, 3676, 3694, 3792, 3808, 3824, 4122, 4146, 4188], 'Maillard': [725, 1068], 'Hamano': [727, 1070], 'Donati': [729, 1072], 'M.A.': [730, 1073], 'Semrad': [731, 1074], 'C.E.': [732, 1075], 'Russell': [733, 1076], 'J.A.': [734, 1077], 'Andreu': [735, 1078], 'A.L.': [736, 1079], 'Vu': [739, 1082], 'T.H.': [740, 1083], 'Tadesse': [741, 1084], 'Nygaard': [743, 863, 1086], 'T.G.': [744, 864, 1087], 'Nonaka': [745, 1088], '2000;': [759, 1102, 4125, 4148, 4190], '47:': [760, 1103], '792-800Crossref': [761, 1104], '(304)': [764, 1107], 'Clinical': [767], 'diagnostic': [768, 3451, 4288], 'tests': [769, 1861, 3462], 'typically': [770], 'reveal': [771], 'leukodystrophy': [772, 1012], 'on': [773, 835, 1013, 1633, 1763, 1769, 1779, 2732, 3730, 3736, 3746, 4253], 'brain': [774, 1014], 'magnetic': [775, 1015], 'resonance': [776, 1016], 'imaging': [777, 1017], 'scans,': [778], 'lactic': [779], 'acidosis,': [780], 'neuropathy': [783], 'demyelination,': [785], 'axonal': [786], 'degeneration,': [787], 'Skeletal': [790], 'biopsies': [792], 'show': [793, 1991, 3022, 3091], 'neurogenic': [794], 'changes': [795], 'together': [796, 3499], 'abnormalities': [799], 'such': [800, 4226], 'ragged': [802], 'red': [803], 'fibers,': [804], 'ultrastructurally': [805], 'abnormal': [806], 'mitochondria,': [807, 4308], 'decreased': [809], 'cytochromec': [810], 'oxidase': [811], 'activity,': [812, 4264], 'isolation': [815], 'association': [818], 'defects.': [824], 'gene,': [833], 'located': [834], 'chromosome': [836], '22q13.32-qter,': [837], 'cause': [840, 926, 3939], '(8Hirano': [843], 'Yebenes': [845], 'Jones': [847], 'A.C.': [848, 3287, 4085], 'Nishino': [849], 'Carlo': [853], 'J.R.': [854], 'Bender': [855], 'A.N.': [856], 'A.F.': [858], 'L.M.': [860], 'Weeks': [861], 'D.E.': [862], 'Am.': [865, 4145], 'Hum.': [867], '63:': [870, 3399], '526-533Abstract': [871], '(95)': [879], '9Nishino': [882, 1110], 'Science.': [888, 939, 1116], '283:': [890, 941, 1118], '689-692Crossref': [891, 942, 1119], '(752)': [894, 945, 1122], 'Normally,': [897, 2542], 'catabolizes': [899], '1-phosphate': [905], '(10Brown': [906, 2513], 'N.S.': [907, 2514], 'Bicknell': [908, 2515], 'Biochem.': [910, 1356, 2517, 2789, 2843, 4169], '334:': [913, 2520], '1-8Crossref': [914, 2521], '(207)': [917, 2524], 'reductions': [928], '(9Nishino': [933], 'report': [949], 'enzymatic': [950, 1952, 1956], 'urine': [961, 1475, 1501, 1510, 1546, 2080, 3166, 3191], 'excretion': [963, 2111, 3149], 'studied': [967, 972], 'at': [968, 1324, 1331, 1396, 1412, 1440, 1610, 1625, 1749, 2463, 2893], 'our': [969, 2006, 3464], 'center.': [970], 'probands': [974, 1043], '29': [977], 'relatives,': [979], '23': [981, 1998], 'The': [984, 1212, 1227, 1343, 1399, 1617, 1929, 2002, 2156, 2218, 2300, 2485, 2501, 2556, 2681, 3054, 3119, 3488, 3761, 3853, 3889], 'clinical': [985, 1959], 'diagnosis': [986, 1130], 'required': [989], 'presence': [991], 'six': [993], 'features': [994], 'follows:': [996], '1)': [997], 'dysmotility;': [1000], '2)': [1001], 'cachexia;': [1002], '3)': [1003], '4)': [1008], '5)': [1011], 'scan;': [1018], '6)': [1020], 'laboratory': [1021, 3465], 'dysfunction.': [1025], 'As': [1026], 'reported': [1027, 2781, 3317, 3344], 'previously,': [1028], 'we': [1029, 1784, 1796, 1845, 2037, 2358, 2380, 2667, 3047], 'homozygous': [1031], 'compound': [1033, 3327], 'heterozygous': [1034, 1045, 1984, 2717], 'gene': [1039, 2490, 3470], '22': [1048], 'relatives': [1049], 'Samples': [1125, 1321], 'three': [1127, 1163, 1688, 1692, 1757, 2198], 'dominant': [1133], 'progressive': [1134], 'external': [1135, 1634], 'ophthalmoplegia,': [1136], 'another': [1137], 'disorder': [1138], 'also': [1146, 1936, 2937], 'studied.': [1147], 'Two': [1148], 'underwent': [1151, 1155, 1162, 2191, 2197], 'hemodialysis.': [1152], 'Patient': [1153], 'A': [1154, 2190, 3262], 'single': [1157], 'hemodialysis,': [1158], 'patient': [1160, 1699, 2189, 2195, 2326], 'B': [1161, 2196], 'consecutive': [1164, 2199], 'weekly': [1165, 2200], 'treatments.': [1166, 2202], 'each': [1168, 1366, 1760, 2872], 'treatment,': [1170, 1189], 'blood': [1171, 1182, 1486, 3334, 3355, 3407, 4022, 4221, 4321], 'samples': [1172, 1487, 1502, 1529, 1597, 2021], 'collected': [1174, 1504], 'immediately': [1175, 1190, 2227], 'before': [1176], 'dialysis,': [1178, 2193, 2242], 'pre-filtered': [1179], 'post-filtered': [1181], '15': [1183], 'min': [1184, 1330], 'after': [1185, 1191, 1203, 2213, 2228, 2241, 3227, 4002, 4023], 'initiation': [1186], 'completion,': [1192], '3': [1194, 1201, 1712, 2239, 3228], 'h,': [1195, 1197, 3229], '1,': [1198], '2,': [1199, 3769], 'days': [1202, 1682, 2254], 'dialysis.': [1204], 'measured': [1206, 1236, 1479, 1624], 'concentration': [1208, 1477, 1823, 2035, 2136, 2143, 2166, 2173, 2262, 2284, 2347, 2394, 2767, 4319], 'these': [1210, 2365, 3461, 4161, 4323], 'samples.': [1211], 'study': [1213, 2496, 3304], 'performed': [1215, 1467, 1832], 'under': [1216], 'protocol': [1218], 'approved': [1219], 'Columbia': [1222], 'University': [1223], 'Institutional': [1224], 'Review': [1225], 'Board.': [1226], 'rate': [1228, 1613, 3974, 4200], 'conversion': [1230, 2559], 'spectrophotometrically': [1237], 'using': [1238, 1425, 1578, 2855], 'modifications': [1239], 'methods': [1241], 'described': [1242], 'previously': [1243, 2780], '(11Friedkin': [1244], 'Roberts': [1246], 'Biol.': [1249, 1282, 3569, 3587, 3603, 3622, 3677, 3695, 3793, 3809, 3825, 4123], 'Chem.': [1250, 1283, 3570, 3588, 3604, 3623, 3678, 3696, 3794, 3810, 3826, 4124], '1954;': [1251], '207:': [1252], '245-256Abstract': [1253], '12Marsh': [1260], 'J.C.': [1261], 'Perry': [1262, 1277], 'Clin.': [1265], 'Invest.': [1266], '1964;': [1267], '43:': [1268], '267-278Crossref': [1269], '(59)': [1272], '13Gallo': [1275], 'R.C.': [1276], 'Breitman': [1279], 'T.R.': [1280], '1967;': [1284], '242:': [1285], '5059-5068Abstract': [1286], 'Buffy': [1293], 'homogenized': [1296], 'lysis': [1298], 'buffer': [1299], '(50': [1300], 'mm': [1301, 1310, 1726, 1734], 'Tris-HCl,': [1302], 'pH': [1303, 1379, 1605], '7.2,': [1304], 'containing': [1305, 1607], '1%': [1306], 'Triton': [1307], 'X-100,': [1308], '2': [1309, 1733, 3857, 3895], 'phenylmethylsulfonyl': [1311], 'fluoride,': [1312], '0.02%': [1314], '2-mercaptoethanol)': [1315], 'subjected': [1317], 'brief': [1319], 'sonication.': [1320], 'centrifuged': [1323, 1489, 1557], '20,000': [1325], '×': [1326, 1428, 1586, 1705, 2401, 2440, 2443, 2450], 'g': [1327], 'for': [1328, 1339, 1393, 1495, 1505, 1842, 1862, 2409, 2478, 2579, 2903, 2939, 3049, 3277, 3416, 3431, 3468, 3547, 3947, 4290], '30': [1329], '4': [1332], '°C,': [1333], 'supernatants': [1336], 'used': [1338, 1512, 1846, 1859, 2128, 2158, 2930], 'assay.': [1342], 'protein': [1344, 1371, 2506, 2544], 'content': [1345], 'determined': [1347, 1424, 2278], 'through': [1348, 3165], 'bicinchoninic': [1350], 'acid': [1351, 1551], 'method': [1352, 2007, 2858], '(14Bradford': [1353], 'M.M.': [1354, 3281, 4079, 4100], 'Anal.': [1355, 2788, 2842], '1976;': [1357, 3624], '72:': [1358], '248-254Crossref': [1359], '(217544)': [1362], 'From': [1365], 'supernatant,': [1367], '100–250': [1368], 'μg': [1369], 'incubated': [1373], '0.1m': [1375], 'Tris': [1376], 'arsenate': [1377], 'buffer,': [1378, 1604], '6.5,': [1380], '10': [1382, 1681, 1703, 2253], 'mmthymidine': [1383], 'total': [1386], 'volume': [1387], '0.1': [1389, 2824], 'ml.': [1390], 'After': [1391], 'incubation': [1392], '1': [1394, 1406, 1725, 1798, 2442, 3868], 'h': [1395, 1455, 2240, 4001], '37': [1397, 1750, 2464], '°C.': [1398], 'reaction': [1400, 2575], 'terminated': [1402], 'addition': [1404], 'ml': [1407, 1713], '0.3m': [1409], 'NaOH.': [1410], 'Absorbance': [1411], '300': [1413], 'nm': [1414], 'measured,': [1416], 'amount': [1419, 1449], 'formed': [1422], '3.4': [1427], '103liter/mol/cm': [1429], 'difference': [1430, 1930], 'molar': [1433], 'extinction': [1434], 'coefficient': [1435, 2044, 2412, 2481], 'alkaline': [1441], 'pH.': [1442], 'Enzyme': [1443], 'expressed': [1446, 2053, 3859], '(nanomoles)': [1452], 'generated': [1453], 'per': [1454, 1456], 'mg': [1457], 'protein.': [1459, 1562], 'Results': [1460], 'obtained': [1462, 2852], 'independent': [1465], 'experiments': [1466], 'duplicate.': [1469], 'Plasma,': [1470, 1540], 'cell': [1471, 1541, 1677, 1761, 3015, 3059, 3097, 3124, 3873, 4074], 'culture': [1472, 1542, 1678, 1684, 2292, 2350, 2397, 2458, 2968, 3005, 3011], 'medium,': [1473, 1543, 2987], 'isocratic': [1482], 'HPLC': [1483, 1496, 1573, 2857], 'method.': [1484], 'Anticoagulated': [1485], 'removed': [1492], 'processed': [1494], 'analysis.': [1497], '24': [1506], 'h.': [1507], 'Aliquots': [1508], 'measure': [1514], 'creatinine': [1517, 1521], 'levels.': [1518, 3760, 3910], 'Simultaneous': [1519], 'serum': [1520], 'measured.': [1527], 'Urine': [1528], 'diluted': [1531, 1545], '1:20': [1532], 'phosphate-buffered': [1534], 'saline': [1535], 'prior': [1536], 'measurement.': [1539], 'treated': [1548], 'perchloric': [1550], '(0.5': [1552], 'm,': [1553], 'final': [1554], 'concentration)': [1555], 'remove': [1559], 'precipitated': [1561], 'Fifty': [1563], 'μl': [1564], 'supernatant': [1567], 'injected': [1569], 'Alliance': [1572], 'apparatus': [1574], 'Waters': [1576], 'Associates,': [1577], 'Alltima': [1580], 'C18': [1581], 'NUC': [1582], '100-Å,': [1583], '5-μm,': [1584], '250': [1585], '4.6-mm,': [1587], 'Alltech': [1588], 'reverse': [1589, 2574], 'phase': [1590], 'column': [1591], 'Alltech,': [1593], '(Deerfield,': [1594], 'IL).': [1595], 'Injected': [1596], 'eluted': [1599, 1621], '50': [1601], 'mmpotassium': [1602], 'phosphate': [1603], '4.0,': [1606], '10%': [1608], 'methanol': [1609], 'flow': [1612], '1.5': [1615, 3337], 'ml/min.': [1616], 'absorption': [1618], 'materials': [1622], '267': [1626], 'nm.': [1627], 'Quantitation': [1628], 'based': [1632, 1643], 'standards.': [1635], 'Definitive': [1636], 'identification': [1637, 2486], 'peak': [1641, 2869], 'retention': [1645, 2896], 'time': [1646, 2290, 2386, 2897, 3051], 'treatment': [1649, 4042], '2nd': [1652, 1765], 'aliquot': [1653, 1799], 'sample': [1656, 2873], 'purified': [1662, 2875], '(from': [1665], 'Sigma)': [1666], 'eliminate': [1668, 3163, 3958, 3990], 'analyzed': [1672, 3182], 'contents': [1675], 'medium': [1679, 1717, 1740, 1801, 1814, 2287, 2351, 2398, 2459], 'over': [1680, 2288], 'plated': [1695, 2447], '105fibroblasts': [1696], 'every': [1698, 1791], 'control': [1701, 1907, 2019, 2073, 2257, 2369, 3029, 3066, 3117], '35': [1704, 2449], '10-mm': [1706, 2451], 'Petri': [1707], 'dishes': [1708, 2452], '(day': [1709], '0)': [1710], 'minimum': [1715, 1738], "Eagle's": [1716, 1739], 'Invitrogen,': [1719], 'plus': [1720], '15%': [1721], 'fetal': [1722], 'bovine': [1723], 'serum,': [1724, 2832], 'pyruvate,': [1727], 'μm': [1729, 2827], 'non-essential': [1730], 'amino': [1731], 'acids,': [1732], 'l-glutamine,': [1735], '1×': [1737], 'vitamin': [1741], 'solution': [1742], 'Invitrogen.': [1744], 'grew': [1746, 2313, 3101, 3126], 'cells': [1748, 1789, 1816, 2344, 2439, 2445, 3081, 3640, 3717], '°C': [1751, 2465], '5%': [1753, 2467], 'CO2.': [1754], 'trypsinized': [1756], 'plates': [1758, 1768], 'line': [1762, 3125], 'day,': [1766, 1775, 1782], '4th,': [1771], '6th,': [1772], '8th': [1774], 'one': [1777, 1811, 2192, 3107, 3122, 3944], 'plate': [1778, 1812], '10th': [1781], 'counted': [1785], 'plate.': [1792], 'Prior': [1793], 'trypsinization,': [1795], 'saved': [1797], 'plates,': [1806], 'without': [1815], 'blank': [1819], 'assess': [1821], 'HPLC.': [1825], 'Statistical': [1826], 'analysis': [1827, 2383], 'data': [1830], 'SPSS': [1834], '10.0': [1835], 'software.': [1836], 'Distributions': [1837], 'values': [1839], 'tested': [1841, 2273], 'normality,': [1843], "Student's": [1847], 't': [1848], 'test': [1849], 'find': [1851], 'differences': [1852], 'groups': [1854], 'distributions.': [1857], 'nonparametric': [1860], 'non-normal': [1863], 'distributions': [1864], 'whenn': [1866], '≤': [1867], '10.': [1868], 'severely': [1875, 3538], '(2': [1881], '±': [1882, 1890, 1917, 1972], '5': [1883], 'nmol/h/mg)': [1884, 1892, 1919], '(667': [1889], '205': [1891], '(p': [1893, 1926], '<': [1894, 1927], '0.001)': [1895], '(Fig.1).': [1896], 'Enzymatic': [1897], '<5%': [1903], 'mean,': [1908], 'they': [1910, 2988, 3168], 'partially': [1912, 2736], 'heterozygotes': [1915, 1934], '(236': [1916], '75': [1918], 'those': [1922, 2883, 3114, 3474, 3721], '0.001).': [1928, 1940], 'statistically': [1937], 'significant': [1938, 2361, 4215], '(p<': [1939], 'There': [1941, 2336], 'no': [1943, 2338], 'relationship': [1944, 3074], 'severity': [1949], 'defect': [1953], 'phenotype.': [1960], 'Thymidine': [1961, 2075], 'dramatically': [1964], '(8.68': [1971], '5.23': [1973], 'μm)': [1974, 2009], '(range': [1975], '3.5–24.7;n': [1976], '=': [1977, 1997, 2016, 2373, 2375], '16),': [1978], 'carriers,': [1987, 2763], 'did': [1989, 2993, 3089], 'not': [1990, 2058, 2754, 2972, 2994, 3090, 3141, 3153, 3162, 3964], 'detectable': [1992, 2730], '(n': [1996, 2015], '14,': [2000], 'respectively).': [2001], 'detection': [2003, 2030], 'limit': [2004, 2031], '(0.05': [2008], 'established': [2011], 'repeating': [2013], 'measurements': [2014], '10)': [2017], 'decreasing': [2023], 'concentrations': [2024, 2066, 2183, 2206, 2698, 2779, 3175, 3187, 3335, 3359, 3422, 3934, 3999, 4219], 'defined': [2028], 'lowest': [2034], 'able': [2039], 'determine': [2041], 'variation': [2046], '(standard': [2047], 'deviation': [2048], 'divided': [2049], 'average': [2051], 'percentage)': [2055], '≤20%': [2056], '(data': [2057], 'shown).': [2059], 'Table': [2060], 'I': [2061], 'shows': [2062, 2204], 'urinary': [2065, 4057], 'subjects.': [2074], 'undetectable': [2077], 'controls,': [2083, 2611], 'whereas': [2084, 2100, 2572, 2612], 'it': [2085, 4009, 4231], 'Both': [2092], 'showed': [2094, 3108, 3632], 'clearances': [2097, 2102, 3199, 3347], 'their': [2101, 2853, 2940, 3208], 'creatinine.Table': [2109], 'IUrinary': [2110], 'MNGIEnPl': [2115], 'thdU': [2116, 2117], 'thdThd': [2118], 'Clμmμmol': [2120], 'Thd/mg': [2121], 'Creat%': [2122], 'Creat': [2123], 'ClMNGIE': [2124], '(I)14.8960.1818.9MNGIE': [2125], '(II)110.34120.4919.1Controls6<0.05<1The': [2126], 'abbreviations': [2127, 2157], 'are:': [2129, 2159], 'Thd,': [2130, 2160], 'thymidine;': [2131, 2161], 'Creat,': [2132, 2162], 'creatinine;': [2133, 2163], 'Pl': [2134, 2164], 'thd,': [2135, 2142, 2165, 2172], 'plasma;': [2140, 2170], 'U': [2141, 2171], 'urine;': [2147, 2177], 'Cl,': [2148, 2178], 'clearance.': [2149, 2179], 'Open': [2150, 2416], 'table': [2151, 2417], 'new': [2154, 2420], 'tab': [2155, 2421], 'Hemodialysis': [2180, 3962], 'circulating': [2182, 2750, 2943, 3174, 3447], 'patients;': [2188], 'dialysis': [2201, 2214, 2229, 3988, 4028], 'Fig.2': [2203], 'before,': [2210], 'during,': [2211], 'both': [2216, 3649], 'reduction': [2219, 2725], 'level': [2223], 'about': [2231], '50%,': [2232], 'indicating': [2233, 2764], 'ultrafiltratable.': [2237], 'However,': [2238, 3226], 'returned': [2248, 3235], 'pretreatment': [2250], 'values.': [2251, 3238], 'Over': [2252], 'culture,': [2256], 'gradually': [2259, 2963], '(Fig.3).': [2276], 'linear': [2280], 'regression': [2281, 2391], '(TableII).': [2299], 'growth': [2301, 2320, 3145], 'curves': [2302], 'varied': [2306], 'considerably.': [2307], 'Fibroblasts': [2308, 3085], 'poorly': [2314], 'controls;': [2318], 'however,': [2319], 'third': [2325, 3106], 'comparable': [2328, 3112], '(Fig.': [2334], '4).': [2335], 'correlation': [2339, 2363, 2411, 2480, 2997, 3026, 3044, 3057], 'fibroblasts;': [2355], 'fact,': [2357], 'found': [2359], 'negative': [2362, 3025], 'variables': [2366], '(−0.714;': [2371], 'p': [2372], '0.001;n': [2374], '18),': [2376], 'which': [2377, 2584, 3031, 3770, 4269], 'disappeared': [2378, 3045], 'when': [2379, 2674, 3046, 3325, 3348], 'adjusted': [2381, 3048], 'culture.Table': [2389], 'IILinear': [2390], 'versus': [2399], 'timeSloperpn(μmol/l/day)': [2400], '102Patient': [2402], '13.70.9590.0036Patient': [2403], '23.20.9460.0046Patient': [2404], '33.60.9010.0376Control': [2405], '1−4.8−0.9320.0076Control': [2406], '2−4.5−0.9760.0015Control': [2407], '3−4.9−0.9790.0016Values': [2408], "Pearson's": [2410, 2479], 'listed': [2414, 2483], 'underr.': [2415, 2484], 'Figure': [2422, 2471], '4Growth': [2423], 'curve': [2424, 3111], 'fibroblasts.': [2427, 3118], 'x': [2428], 'axis': [2429, 2435], 'day': [2431], 'culture.': [2433, 3053, 3147], 'y': [2434], '10−3.': [2441], '105': [2444], 'grown': [2454], 'standard': [2457], '(see': [2460], '“Experimental': [2461], 'Procedures”)': [2462], 'CO2.View': [2468], 'Large': [2469], 'Image': [2470], 'ViewerDownload': [2472], 'Hi-res': [2473], 'image': [2474], 'Download': [2475], '(PPT)': [2476], 'Values': [2477], 'loss-of-function': [2488], 'has': [2492, 2507, 2729], 'enabled': [2493], 'us': [2494, 3069], 'pathogenesis': [2498], 'functional': [2502, 2672], 'significance': [2503], 'characterized': [2510], 'vitro': [2512, 2947], 'homodimer': [2530, 2635], 'important': [2533, 2565, 2578], 'role': [2534], 'homeostasis': [2537], 'cellular': [2539], 'pools.': [2541], 'forward': [2557], 'reaction,': [2558], 'thymine,': [2563], 'catabolism': [2568], 'nucleoside,': [2571], 'pyrimidine': [2581], 'salvage': [2582, 3528, 3733, 3748], 'pathway,': [2583, 3749], 'allows': [2585], 'converted': [2589], '(TTP),': [2593], 'essential': [2595], 'building': [2596], 'block': [2597], 'DNA.': [2599], 'carriers': [2617, 2715, 2742, 4162, 4210], '∼35%': [2619], 'value.': [2623], 'This': [2624, 2773], 'result': [2625, 2774, 2881], 'consistent': [2627], 'fact': [2630, 3078], '(15Kubilus': [2636], 'Lee': [2638, 3388], 'L.D.': [2639], 'Baden': [2640], 'H.P.': [2641], '1978;': [2645], '527:': [2646], '221-228Crossref': [2647], '(21)': [2650], '16Miyazono': [2653], 'K.': [2654], 'Heldin': [2655], 'C.H.': [2656], 'Biochemistry.': [2657], '1989;': [2658], '28:': [2659], '1704-1710Crossref': [2660], '(48)': [2663], 'Scholar),': [2665], 'would': [2668], 'expect': [2669], '25%': [2670], 'only': [2675, 2721, 2973, 3472], '50%': [2676], 'monomers': [2678], 'normal.': [2680], 'direct': [2683], 'consequence': [2684, 4259], 'this': [2686, 3177, 3201, 3326, 3417, 3548, 3744, 4291], 'loss': [2687, 3130, 4261], 'seems': [2690], 'accumulation': [2694], 'Plasma': [2697, 3212], 'elevated': [2702, 4300], '60-fold': [2705], 'mutations.': [2719], 'Therefore,': [2720], 'complete': [2722], 'near-total': [2724], 'consequences': [2731, 4249], 'metabolism,': [2734], 'sufficient': [2744], 'maintain': [2746], 'low': [2747, 3173, 3205, 3244, 3318], 'could': [2753, 2901, 2936, 3753, 4211], 'detect': [2755], 'less': [2769], '0.05': [2771], 'μm.': [2772, 3338], 'contrasts': [2775], '(17Dudman': [2782, 2836], 'N.P.': [2783, 2837], 'Deveski': [2784, 2838], 'W.B.': [2785, 2839], 'Tattersall': [2786, 2802, 2840, 2914], 'M.H.': [2787, 2803, 2841, 2915], '1981;': [2790, 2844], '115:': [2791, 2845], '428-437Crossref': [2792, 2846], '(22)': [2795, 2849], '18Holden': [2798], 'Hoffbrand': [2800, 2912], 'A.V.': [2801, 2913], 'Eur.': [2804, 2916], 'Cancer.': [2806, 2918, 4189], '1980;': [2807, 2919], '16:': [2808, 2920], '115-121Abstract': [2809, 2921], '(38)': [2815, 2927], 'Scholar)': [2817, 2851, 2929, 3316, 3376, 3631, 3705], 'ranging': [2818], '<0.04': [2820], '0.6': [2822], '1.16': [2826], 'respectively.': [2833], 'Dudmanet': [2834], 'al.': [2835, 2909], 'results': [2854, 3436, 3724], 'UV': [2860], 'detection.': [2861], 'verified': [2863], 'specificity': [2865], 'treating': [2871], 'comparing': [2879], 'untreated': [2885], 'samples,': [2886], 'order': [2888, 4244], 'identify': [2890, 2995, 3454], 'peaks,': [2891], 'co-eluting': [2892], 'same': [2895], 'thymidine,': [2899, 3137, 3498, 4268], 'account': [2902, 2938], 'falsely': [2904], 'concentrations.': [2906, 2945], 'Holden': [2907], 'et': [2908], '(18Holden': [2910], 'radioimmunoassay;': [2932], 'therefore,': [2933, 4313], 'nonspecific': [2934], 'signals': [2935], 'overestimation': [2941], 'studies': [2948], 'provides': [2951], 'further': [2952], 'Normal': [2961, 3159], 'catabolize': [2964], 'medium.': [2969, 3012], 'unable': [2977], 'degrade': [2979], 'initially': [2983], 'even': [2989], 'release': [2990], 'These': [3013, 3723], 'variables,': [3014], 'level,': [3019], 'seemed': [3020], 'clear': [3024], 'group,': [3030], 'actually': [3033], 'effect': [3037, 3550], 'covariate,': [3040], 'time,': [3041], 'lack': [3055, 3493], 'prevented': [3068], 'identifying': [3071], 'any': [3072], 'quantitative': [3073], 'derived': [3075], 'contains': [3082], 'TP.': [3084], 'homogeneous': [3093], 'behavior;': [3095], 'lines': [3098], 'very': [3102, 3172, 3204, 3969], 'poorly,': [3103], 'but': [3104, 3196, 3413], 'observation': [3120], 'indicates': [3128, 3253], 'function': [3133, 4159], '≤0.90': [3138], 'μm,': [3139], 'do': [3140, 3161], 'interfere': [3142], 'fibroblast': [3144], 'Urinary': [3148], 'completely': [3154], 'effective': [3155, 3970, 4033], 'absent': [3170], 'nucleoside.': [3178], 'excreted': [3185], '(96': [3192], '412': [3194], 'μm),': [3195], 'creatinine.': [3211], 'similarly': [3221], 'freely': [3223], 'dialyzable': [3224], 'molecules.': [3225], 'pre-dialysis': [3237, 3998], 'clearance,': [3246], 'reabsorbed.': [3261], 'Na+/thymidine': [3263], 'transporter': [3264], 'brush': [3269], 'border': [3270], 'proximal': [3273], 'tubule,': [3274], 'responsible': [3276], 'reabsorption': [3279, 3428, 4047, 4203], '(19Gutierrez': [3280, 4078], 'Brett': [3282, 4080], 'C.M.': [3283, 4081, 4121], 'Ott': [3284, 4082], 'R.J.': [3285, 4083], 'Hui': [3286, 4084], 'Giacomini': [3288, 4086, 4101], 'K.M.': [3289, 4087, 4102], '1992;': [3293, 4091], '1105:': [3294, 4092], '1-9Crossref': [3295, 4093], '(66)': [3298, 4096], 'Interestingly,': [3301], 'previous': [3303, 3502], '(20Ensminger': [3305], 'W.D.': [3306], 'Frei': [3307], 'III,': [3308], 'Cancer': [3310, 3370, 3395], 'Res.': [3311, 3371], '1977;': [3312], '37:': [3313], '1857-1863PubMed': [3314], 'humans': [3324, 3361], 'infused': [3329, 3351], 'intravenously': [3330], 'induce': [3332], 'steady-state': [3333, 3354], 'around': [3336], 'other': [3341], 'papers': [3342], 'elevate': [3353], 'millimolar': [3358], '(21Zaharko': [3362], 'D.S.': [3363, 3381], 'Bolten': [3364, 3382], 'B.J.': [3365, 3383], 'Chiuten': [3366], 'Wiernik': [3368], 'P.H.': [3369], '1979;': [3372, 3398], '39:': [3373], '4777-4781PubMed': [3374], 'monkeys': [3379], '(22Zaharko': [3380], 'Kobayashi': [3384], 'Blasberg': [3386], 'R.G.': [3387], 'S.S.': [3389], 'Giovanella': [3390], 'B.C.': [3391], 'Stehlin': [3392], 'Jr.,': [3393], 'J.S.': [3394], 'Treat.': [3396], 'Rep.': [3397], '945-949PubMed': [3400], 'micromolar': [3411], 'range,': [3412], 'low.': [3420], 'Millimolar': [3421], 'probably': [3425, 3776, 4233], 'saturate': [3426], 'mechanism,': [3429], 'accounting': [3430], 'clearances.': [3434], 'Our': [3435], 'indicate': [3437, 3725], 'powerful': [3450], 'tools': [3452], 'currently': [3459], 'performing': [3460], 'look': [3467], 'whose': [3476], 'disturbed': [3483], 'biochemical': [3486], 'assays.': [3487], 'facts': [3489], 'accumulate': [3497, 4267], 'observations': [3503], 'alterations': [3506, 3524, 3757, 3780], 'dysfunction-like': [3509], 'symptoms,': [3510], 'suggest': [3511], 'impairment.': [3520], 'pathway': [3529, 3734], 'seem': [3530, 3965], 'affect': [3532], '(dNTP)': [3535], 'mitochondria': [3540, 3553, 3727], 'nuclei.': [3543], 'One': [3544], 'possible': [3545, 3945, 4011], 'reason': [3546], 'selective': [3549], 'thought': [3555], 'separate': [3558], 'independently': [3560], 'regulated': [3561], 'dNTP': [3562, 3784, 3905, 3916], '(23Bestwick': [3564, 3672], 'R.K.': [3565, 3581, 3673, 3689, 3787], 'Mathews': [3566, 3584, 3674, 3692, 3790], 'C.K.': [3567, 3585, 3675, 3693, 3791], '1982;': [3571, 3589, 3679, 3697, 3795], '257:': [3572, 3590, 3680, 3698, 3796], '9305-9308Abstract': [3573, 3681], '24Bestwick': [3580, 3688], 'Moffett': [3582, 3690, 3788], 'G.L.': [3583, 3691, 3789], '9300-9304Abstract': [3591, 3699, 3797], '25Berk': [3598, 3804], 'A.J.': [3599, 3805], 'Clayton': [3600, 3616, 3619, 3806], 'D.A.': [3601, 3620, 3807, 4164], '1973;': [3605, 3811], '248:': [3606, 3812], '2722-2729Abstract': [3607, 3813], 'Bogenhagen': [3614], '(26Bogenhagen': [3617], '249:': [3625], '2938-2944Abstract': [3626], 'replication': [3636], 'mouse': [3638], 'L': [3639], 'resistant': [3642], 'inhibition': [3644], 'methotrexate': [3646], '5-flurodeoxyuridine;': [3648], 'agents': [3650], 'blockde': [3651], 'novo': [3652, 3739], 'synthesis': [3654], 'inhibiting': [3656, 4208], 'thymidylate': [3657, 3663], 'synthetase': [3658], 'converts': [3660, 3771], 'dUMP': [3661], '(deoxythymidine': [3664], 'monophosphate': [3665], 'dTMP).': [3667], 'Subsequently,': [3668], 'Bestwick': [3669], 'colleagues': [3671], 'expansion': [3708], 'response': [3719], 'anti-metabolites.': [3722], 'depend': [3728], 'de': [3738], 'synthetic': [3740], 'pathway.': [3741], 'dependence': [3745], 'dTTP': [3751], 'perturbed': [3755], 'existence': [3762], 'specific': [3766], 'kinase': [3768, 3856, 3867, 3894], 'monophosphate,': [3775], 'contributes': [3777], 'pool': [3785], '(24Bestwick': [3786], '27Johansson': [3820], 'Karlsson': [3822], '1997;': [3827], '13:': [3828], '8454-8458Abstract': [3829], '(102)': [3836], '28Arnér': [3839], 'E.S.J.': [3840, 3876], 'Eriksson': [3841, 3877], 'Pharmacol.': [3843, 3879, 4170], 'Ther.': [3844, 3880], '67:': [3846, 3882], '155-186Crossref': [3847, 3883], '(514)': [3850, 3886], 'constitutively,': [3860], 'contrast': [3862], 'cytosolic': [3865], 'up-regulated': [3871], 'during': [3872], 'division': [3874], '(28Arnér': [3875], 'constant': [3890], 'contribute': [3899], 'susceptibility': [3902], 'lead': [3921, 4212], 'If': [3932, 4225], 'impairment,': [3943], 'therapy': [3946], 'will': [3949], 'or,': [3953], 'if': [3954], 'possible,': [3955, 4242], 'virtually': [3957], 'blood.': [3961, 4275], 'does': [3963], 'strategy,': [3971], 'production': [3976], 'exceed': [3983], 'ability': [3985], 'molecule': [3992], 'blood,': [3995], 'restoring': [3996], '<3': [4000], 'end': [4004], 'treatment.': [4007], 'Alternatively,': [4008], 'body': [4014], 'stores': [4015], 'rapidly': [4019], 'into': [4021], 'treatments': [4029], 'ultimately': [4031], 'removing': [4035], 'accumulated': [4036], 'Another': [4041], 'strategy': [4043, 4228], 'blocking': [4045], 'kidney,': [4053], 'thereby': [4054], 'increasing': [4055], 'elimination': [4058], 'Several': [4061], 'descriptions': [4062], 'kinetics': [4065], 'pharmacological': [4067], 'properties': [4068], 'transporters': [4071], 'published': [4077], '29Gutierrez': [4099], '1993;': [4106], '1149:': [4107], '202-208Crossref': [4108], '(65)': [4111], '30Ward': [4114], 'J.L.': [4115], 'Sherali': [4116], 'Mo': [4118], 'Z.P.': [4119], 'Tse': [4120], '275:': [4126], '8375-8381Abstract': [4127], '(253)': [4135], '31Van': [4138], 'Aubel': [4139], 'R.A.': [4140], 'Masereeuw': [4141], 'Russel': [4143], 'F.G.': [4144], 'Physiol.': [4147], '279:': [4149], 'F216-F232Crossref': [4150], 'Multiple': [4154], 'drugs': [4155], 'inhibit': [4157], '(32Griffith': [4163], 'Conant': [4165], 'A.R.': [4166], 'Jarvis': [4167], 'S.M.': [4168], '1990;': [4171], '40:': [4172], '2297-2303Crossref': [4173], '(17)': [4176], '33Smith': [4179], 'P.G.': [4180], 'Marshman': [4181], 'Newell': [4183], 'D.R.': [4184], 'Curtin': [4185], 'N.J.': [4186], 'Br.': [4187], '82:': [4191], '924-930Crossref': [4192], '(31)': [4195], 'Decreasing': [4198], 'chemically': [4207], 'decrease': [4216], 'effective,': [4230], 'necessary': [4234], 'treat': [4236], 'early': [4240], 'avoid': [4246], 'probable': [4247], 'irreversible': [4248], 'toxicity': [4252], 'cells.': [4254], 'summary,': [4256], 'cannot': [4270], 'efficiently': [4272], 'eliminated': [4273], 'measurement': [4278], 'value': [4289], 'disease.': [4292], 'that,': [4295], 'persistently': [4299], 'unbalances': [4304], 'abnormalities;': [4312], 'therapeutic': [4314], 'strategies': [4315], 'effective.': [4327]}, 'cited_by_api_url': 'https://api.openalex.org/works?filter=cites:W2169143920', 'counts_by_year': [{'year': 2024, 'cited_by_count': 3}, {'year': 2023, 'cited_by_count': 4}, {'year': 2022, 'cited_by_count': 5}, {'year': 2021, 'cited_by_count': 10}, {'year': 2020, 'cited_by_count': 13}, {'year': 2019, 'cited_by_count': 12}, {'year': 2018, 'cited_by_count': 9}, {'year': 2017, 'cited_by_count': 6}, {'year': 2016, 'cited_by_count': 12}, {'year': 2015, 'cited_by_count': 5}, {'year': 2014, 'cited_by_count': 8}, {'year': 2013, 'cited_by_count': 2}, {'year': 2012, 'cited_by_count': 6}], 'updated_date': '2025-01-11T07:06:14.657600', 'created_date': '2016-06-24'}
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