Publication Information

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1250, 1265, 1277, 1315, 1322, 1365, 1372, 1419, 1478, 1506, 1530, 1561, 1566, 1574, 1597, 1624, 1630, 1663, 1675, 1683, 1699, 1708, 1725, 1770, 1951, 1964, 2029, 2048, 2051, 2057, 2077, 2080, 2123, 2136, 2206, 2218, 2230, 2266, 2272, 2276, 2287, 2330, 2348, 2361, 2377, 2382, 2415, 2430, 2439, 2477, 2486, 2522, 2534, 2661, 2681, 2700, 2745, 2760, 2801, 2839, 2860, 2900, 2905, 2924, 2934, 2940, 2948, 2956, 2961, 3005, 3102, 3107, 3118, 3130, 3142, 3148, 3234, 3244, 3247, 3257, 3285, 3389, 3401, 3407, 3419, 3426, 3516, 3528, 3555, 3568, 3576, 3597, 3601, 3619, 3627, 3638, 3657, 3667, 3672, 3696, 3716, 3724, 3732, 3743, 3754, 3763, 3766, 3777, 3782, 3807, 3812, 3827, 3834, 3842, 3857, 3859, 3865, 3887, 3891, 3939, 3944, 3959, 3969, 3974, 3989, 3998, 4001, 4023, 4029, 4068, 4081, 4088, 4097, 4113, 4122, 4132, 4147, 4227, 4233, 4236, 4241, 4268, 4273, 4295, 4299], 'pathogenesis': [16, 291], 'of': [17, 29, 41, 51, 57, 135, 156, 173, 187, 228, 231, 234, 252, 271, 292, 304, 316, 326, 332, 410, 431, 448, 462, 503, 506, 509, 527, 546, 592, 632, 674, 680, 699, 956, 973, 1144, 1157, 1222, 1237, 1364, 1374, 1480, 1492, 1508, 1523, 1525, 1568, 1599, 1626, 1701, 1707, 1710, 1727, 1924, 1926, 1944, 1979, 2050, 2079, 2113, 2138, 2208, 2268, 2289, 2363, 2366, 2379, 2384, 2417, 2432, 2441, 2479, 2524, 2526, 2791, 2859, 2920, 2963, 3026, 3144, 3303, 3317, 3329, 3361, 3400, 3428, 3562, 3570, 3621, 3629, 3650, 3742, 3753, 3765, 3836, 3890, 3899, 3953, 3961, 4055, 4070, 4090, 4238, 4240, 4251, 4270, 4272, 4301], 'Huntington': [18, 179, 293, 454, 550, 558], 'disease.': [19, 294, 627], 'Nonetheless,': [20, 295], 'specific': [22, 297], 'defects': [24, 299], 'due': [25, 153, 300, 428, 609], 'to': [26, 154, 301, 429, 610, 847, 1325, 1335, 1476, 1504, 1520, 1689, 1949, 1982, 2075, 2127, 2180, 2205, 2237, 2244, 2251, 2259, 2265, 2446, 2540, 2546, 2579, 2593, 2697, 2914, 2929, 3114, 3137, 3243, 3268, 3386, 3552, 3595, 3654, 3796, 4067, 4087, 4096, 4121, 4249, 4306], 'presence': [28, 251, 303, 526, 2383, 2431, 3628], 'mutant': [30, 42, 89, 235, 253, 305, 317, 364, 510, 528, 668, 1054, 1209, 1266, 1316, 1381, 1443, 1481, 1509, 1537, 1583, 1711, 1772, 1777, 3668, 3778, 3808, 3843, 3970, 4024, 4082, 4100, 4234, 4265], 'huntingtin': [31, 43, 75, 236, 254, 306, 318, 350, 511, 529, 669, 1055, 1210, 1382, 1448, 1482, 1510, 1584, 1712, 1768, 1778], 'have': [32, 307, 860, 1044, 1190, 1255, 1273, 1540, 1796, 4107], 'not': [33, 152, 266, 308, 427, 541, 1595, 2577, 3009, 3573, 3737, 3862, 4117, 4129], 'been': [34, 309, 778, 1045, 1274, 1797], 'fully': [35, 310], 'elucidated.': [36, 311], 'To': [37, 219, 312, 494, 2831, 3069, 3178, 3748, 3911, 4224], 'determine': [38, 313, 2832, 2930, 3070, 3179, 3596, 3912], 'effects': [40, 230, 315, 505, 1479, 1507, 1709], 'on': [44, 237, 319, 512, 1483, 1511, 1713, 2690], 'production,': [47, 54, 322, 329, 1496, 1591, 3182], 'thorough': [49, 324, 1490], 'analysis': [50, 227, 325, 502, 1491, 3264], 'respiration,': [52, 327, 2184], 'ATP': [53, 82, 139, 257, 328, 357, 414, 532, 851, 1495, 1546, 3027, 3030, 3034, 3071, 3110, 3149, 3181, 3346, 3871, 3875, 3905, 3909, 3913, 3921, 3941, 3955, 3963], 'functioning': [56, 331], 'respiratory': [59, 158, 239, 334, 433, 514, 571, 865, 1498, 1527, 1562, 1600, 1664, 2053, 2139, 2212, 2273, 2290, 2761, 3209, 3408, 3717, 3744, 3751, 3990, 4243, 4275, 4312], 'complexes': [60, 189, 335, 464, 866, 1528, 1627, 1667, 2296, 4244], 'was': [61, 102, 110, 128, 146, 151, 336, 377, 385, 403, 421, 426, 658, 882, 1501, 1571, 1933, 1947, 1968, 1993, 2059, 2073, 2120, 2133, 2168, 2178, 2186, 2203, 2224, 2278, 2284, 2317, 2358, 2374, 2412, 2424, 2450, 2473, 2518, 2544, 2663, 2683, 2705, 2785, 2927, 2945, 2969, 3008, 3197, 3241, 3251, 3265, 3587, 3609, 3614, 3663, 3713, 3773, 3854, 3965, 4063, 4078, 4128, 4245, 4309], 'carried': [62, 337, 1502, 2425, 4316], 'out': [63, 338, 1503, 2426], 'clonal': [65, 340, 1438], 'striatal': [66, 90, 341, 365, 775, 1192, 1235, 1268, 1439, 1677, 1728, 1741, 3602], 'cells': [67, 91, 96, 342, 366, 371, 1254, 1279, 1284, 1317, 1324, 1367, 1379, 1538, 1552, 1866, 1901, 1972, 2031, 2083, 2115, 2210, 2270, 2741, 3073, 3183, 3275, 3372, 3437, 3577, 3590, 3669, 3779, 3784, 3809, 3814, 3844, 3971, 3976, 4071, 4083, 4091, 4101, 4115, 4266], 'established': [68, 343, 1191], 'from': [69, 344, 875, 1141, 1195, 1733, 1752, 1784, 2800, 2955, 3124, 4000], 'HdhQ7': [70, 345], '(wild-type)': [71, 346, 3368, 3893, 4048, 4156], 'HdhQ111': [73, 348], '(mutant': [74, 349], 'knock-in)': [76, 351], 'mouse': [77, 352], 'embryos.': [78, 353], 'Mitochondrial': [79, 354, 3874, 4031], 'respiration': [80, 109, 137, 273, 355, 384, 412, 548, 2088, 2167, 2195, 2219, 2325, 2944, 2968, 3198, 3586, 3608, 3631, 3662, 3729, 3758, 3771, 3792, 3840, 4228, 4293, 4302], 'were': [84, 163, 359, 438, 1318, 1332, 1556, 1672, 1732, 1747, 1756, 1782, 1828, 1867, 1881, 1902, 1973, 2032, 2084, 2263, 2327, 2344, 2350, 2576, 2591, 2649, 2677, 2742, 2780, 2798, 2912, 2953, 3019, 3036, 3074, 3093, 3104, 3112, 3122, 3184, 3277, 3282, 3334, 3349, 3373, 3633, 3646, 3693, 3720, 3736, 3746, 3760, 3794, 3803, 3824, 3861, 3918, 3986, 4019, 4043, 4260, 4315], 'significantly': [85, 360, 1541, 3664, 3738, 3774, 3804, 3966, 4020], 'reduced': [86, 361, 1542, 2480, 3665, 3805, 3837, 4231], 'compared': [92, 367, 1281, 1549, 3636, 3670, 3780, 3810, 3972, 4027], 'with': [93, 368, 961, 1282, 1368, 1390, 1550, 1606, 1779, 1838, 1975, 2066, 2427, 2482, 2603, 2768, 2923, 3015, 3201, 3375, 3592, 3671, 3781, 3811, 3973, 3997, 4028], 'wild-type': [95, 370, 1201, 1323, 1446, 1551, 1785, 3673, 3783, 3813, 3975, 4114, 4263], 'when': [97, 117, 141, 372, 392, 416, 1280, 1388, 3611, 3674, 3699, 3740, 3815, 3977, 4085], 'either': [98, 142, 373, 417, 3675, 3816, 3978], 'glutamate/malate': [99, 143, 374, 418], 'or': [100, 144, 261, 375, 419, 536, 958, 1445, 1614, 2155, 2255, 2301, 2767, 3218, 3340, 3435, 3684, 3820, 3982, 4015, 4072], 'succinate': [101, 145, 376, 420, 2148, 3219, 3676, 3817, 3979, 4012], 'used': [103, 129, 147, 378, 404, 422, 555, 1436, 1757, 2074, 2105, 2328, 2592, 2928, 3020, 3105, 3136, 3146, 3290, 3379, 3413, 3694, 3714, 3795, 3825, 3855, 3987], 'as': [104, 130, 148, 379, 405, 423, 1749, 1883, 1995, 2016, 2311, 2360, 2452, 2485, 2533, 2600, 2708, 2804, 2939, 2960, 2979, 3042, 3344, 3695, 3715, 3826, 3845, 3856, 3923, 3988], 'substrate.': [106, 132, 381, 407], 'However,': [107, 382, 1554, 3566, 3698], 'similar': [111, 386, 1572, 4140], 'two': [114, 389, 1531, 1575, 3639, 3733, 3755, 3866, 3945, 4133, 4148, 4296], 'cell': [115, 167, 390, 442, 1042, 1193, 1206, 1220, 1239, 1330, 1341, 1376, 1423, 1440, 1532, 1576, 1744, 1763, 1774, 1794, 3125, 3640, 3725, 3734, 3756, 3867, 3916, 3946, 4025, 4134, 4149, 4297], 'lines': [116, 391, 1139, 1194, 1331, 1424, 1441, 1795, 2903, 2922, 3641, 3735, 3868, 3947, 4135, 4298], 'artificial': [119, 394, 3704], 'electron': [120, 395, 2487, 2535, 3705], 'donor': [121, 396, 3706], 'TMPD/ascorbate,': [122, 397], 'which': [123, 398, 672, 879, 1746, 2174, 2185, 2943, 2967, 3397], 'feeds': [124, 399, 3708], 'into': [125, 400, 3418, 3681, 3690, 3710], 'complex': [126, 401, 967, 1338, 1499, 1563, 1696, 2239, 2246, 2253, 2261, 2306, 2337, 2453, 2548, 2581, 2597, 2916, 2935, 2949, 2964, 3292, 3306, 3682, 3691, 3711, 4304], 'IV,': [127, 402], 'The': [133, 408, 1204, 1761, 1865, 2372, 2422, 2443, 2537, 2918, 3191, 3769, 4053], 'attenuation': [134, 409], 'substrate': [150, 425, 2213], 'complexes,': [159, 434], 'because': [160, 435], 'their': [161, 436, 3900], 'activities': [162, 437, 1500, 1625, 2343, 4042, 4153, 4305], 'equivalent': [164, 439], 'both': [166, 441, 1329], 'lines.': [168, 443, 1533, 1577, 3726], 'Intriguingly,': [169, 444], 'striatum': [172, 447, 701, 975, 1143, 1226, 1631, 1684], 'presymptomatic': [174, 449, 781, 1613], 'pathological': [176, 451, 590], 'grade': [177, 452, 1615], '1': [178, 453, 1927, 1960], 'disease': [180, 455, 551, 709], 'cases': [181, 456, 783, 877], 'there': [182, 457, 1260, 1555, 1619, 3719, 3933], 'also': [184, 459, 861, 3513], 'no': [185, 460, 1557, 1621, 3721, 3935], 'I–IV': [190, 465, 1628], '(Guidetti,': [191, 466], 'P.,': [192, 467], 'Charles,': [193, 468], 'V.,': [194, 469], 'Chen,': [195, 470], 'E.': [196, 471, 987, 997, 1013, 1108, 1176, 1290, 1292, 1462, 1813, 1999, 2001], 'Y.,': [197, 472], 'Reddy,': [198, 473], 'P.': [199, 474, 795, 941, 1015, 1100, 1168, 1454, 1633, 1805, 2614, 2724], 'H.,': [200, 203, 475, 478], 'Kordower,': [201, 476], 'J.': [202, 477, 721, 791, 800, 1004, 1061, 1130, 2815, 3050, 3446, 3462, 3534], 'Whetsell,': [204, 479], 'W.': [205, 480], 'O.,': [206, 481], 'Jr.,': [207, 482, 1643], 'Schwarcz,': [208, 483], 'R.,': [209, 484], 'Tagle,': [211, 486], 'D.': [212, 487, 799, 1098, 1166, 1452, 1803, 2397, 2503, 2562, 2606, 2728, 2874], 'A.': [213, 488, 785, 797, 1021, 1032, 2610, 2612, 2888, 2992, 3190, 3501, 3532], '(2001)': [214, 489], 'Exp.': [215, 490, 1649], 'Neurol.': [216, 491, 723, 741, 897, 923, 1650], '169,': [217, 492], '340–350).': [218, 493], 'our': [220, 495, 1705], 'knowledge,': [221, 496], 'this': [222, 497, 1238, 1433, 1593, 1702, 1759, 2040, 3154, 3195, 3272], 'first': [225, 500], 'comprehensive': [226, 501], 'physiological': [232, 507], 'levels': [233, 508, 818, 1272, 3032, 3111, 3525, 3906, 3942], 'function': [240, 270, 515, 545], 'within': [241, 516], 'an': [242, 517, 582, 2035, 3202], 'appropriate': [243, 518, 1426], 'cellular': [244, 519], 'context.': [245, 520], 'These': [246, 521, 1679, 3604, 3929], 'findings': [247, 522, 1579, 1680, 3999], 'demonstrate': [248, 523, 1417, 3931], 'impairs': [255, 530], 'through': [259, 534, 1596, 3396], 'one': [260, 535], 'more': [262, 537, 1319], 'mechanisms': [263, 538, 1156], 'do': [265, 540], 'directly': [267, 542], 'affect': [268, 543], 'complexes.': [274, 549, 1601, 4276], '(HD)': [552], '1The': [553], 'abbreviations': [554], 'are:': [556], 'HD,': [557, 1233], 'disease;': [559], '3-NP,': [560], '3-nitropropionic': [561, 962], 'acid;': [562, 576], 'TMPD,': [563], 'N,N,N′,N′-tetramethylphenylenediamine': [564, 2160], 'dihydrochloride;': [565], 'FCCP,': [566], 'carbonyl': [567, 578, 2197], 'cyanide': [568, 579, 2198], '4-(trifluoromethoxy)phenylhydrazone;': [569], 'RCR,': [570], 'control': [572], 'ratio;': [573], 'MOPS,': [574, 2750], '3-(N-morpholino)propanesulfic': [575], 'CCCP,': [577, 2756], 'm-chlorophenylhydrazone.': [580], 'autosomal': [583], 'dominant': [584], 'neurodegenerative': [585], 'disorder': [586], 'caused': [587, 970, 1052], 'by': [588, 853, 1053, 1384, 1922, 2135, 2286, 2376, 2414, 2438, 2475, 2520, 2936, 2947, 3284, 3355], 'expansion': [591], 'CAG': [593], 'repeats': [594], 'gene': [597, 634], 'encoding': [598], 'for': [599, 677, 849, 1152, 1244, 1428, 1666, 1959, 2107, 2129, 2170, 2188, 2226, 2280, 2295, 2305, 2319, 2329, 2596, 2654, 2763, 2862, 2871, 2933, 3021, 3089, 3099, 3147, 3153, 3194, 3253, 3380, 3425, 3600, 3656], 'protein': [601, 3115, 3139, 3280, 4073, 4110, 4123, 4144], 'called': [602], 'huntingtin.': [603], 'Disease': [604, 642], 'symptomatology': [605, 980], 'progression': [607], 'are': [608, 1148, 1425, 1620, 2978, 3564, 4230], 'massive': [611], 'neuronal': [612, 880, 1742], 'death': [615, 1342], 'striatum,': [618], 'cerebral': [622], 'cortex': [623, 821], 'later': [624], 'Even': [628], 'though': [629], 'identification': [631], 'contains': [636], 'disease-causing': [638], 'mutation': [639], '(1The': [640], "Huntington's": [641], 'Collaborative': [643], 'Research': [644], 'Group': [645], 'Cell.': [646], '1993;': [647, 834, 1006], '72:': [648], '971-983Abstract': [649], 'Full': [650, 2821, 2823, 3452], 'Text': [651, 2822, 2824, 3453], 'PDF': [652, 2825, 3454], 'PubMed': [653, 727, 745, 837, 901, 927, 948, 1009, 1036, 1066, 1090, 1117, 1135, 1185, 1309, 1357, 1406, 1471, 1654, 1822, 1897, 2009, 2465, 2622, 2641, 2826, 2892, 2996, 3055, 3455, 3468, 3482, 3505, 3543], 'Scopus': [654, 728, 746, 902, 928, 949, 1037, 1067, 1091, 1118, 1186, 1310, 1358, 1407, 1472, 1655, 1823, 2010, 2466, 2623, 2642, 2827, 2893, 2997, 3056, 3469, 3483, 3506, 3544], '(7279)': [655], 'Google': [656, 730, 748, 772, 806, 838, 904, 930, 951, 1010, 1039, 1069, 1093, 1120, 1136, 1188, 1312, 1360, 1409, 1474, 1657, 1825, 1898, 2012, 2468, 2625, 2644, 2829, 2895, 2999, 3058, 3456, 3471, 3485, 3508, 3546], 'Scholar)': [657, 1189, 1475, 2013, 3059], 'fundamental': [660], 'discovery,': [661], 'it': [662, 1242, 3520, 3550], 'still': [664], 'unclear': [665], 'how': [666], 'causes': [670], 'pathogenesis,': [671], 'essential': [675], 'importance': [676], 'development': [679], 'successful': [681], 'treatment': [682, 955, 1363], 'strategies.': [683], 'impaired': [692, 703, 850], 'HD.': [694, 1223], 'Positron': [695], 'emission': [696], 'tomography': [697], 'scans': [698], 'showed': [702], 'glucose': [704], 'metabolism': [705], 'early': [706], '(2Kuhl': [710], 'D.E.': [711, 733, 751], 'Phelps': [712, 758], 'M.E.': [713, 759, 1110, 1178, 1302, 1348, 1397, 1464, 1815], 'Markham': [714, 738, 752], 'C.H.': [715, 739, 753], 'Metter': [716, 734, 754], 'E.J.': [717, 735, 755, 2628], 'Riege': [718, 736, 756], 'W.H.': [719, 737, 757], 'Winter': [720], 'Ann.': [722, 740, 896, 922], '1982;': [724, 3465], '12:': [725, 1307], '425-434Crossref': [726], '(363)': [729], 'Scholar,': [731, 749, 905, 931, 1011, 1070, 1094, 1121, 2626, 3457, 3472, 3486], '3Kuhl': [732], '1984;': [742], '15:': [743], 'S119-S125Crossref': [744], '(118)': [747], '4Kuhl': [750], 'Mazziotta': [760], 'J.C.': [761], 'Res.': [762, 765, 2617], 'Publ.': [763], 'Assoc.': [764], 'Nerv.': [766], 'Ment.': [767], 'Dis.': [768], '1985;': [769], '63:': [770], '199-209PubMed': [771], 'Scholar).': [773, 807, 952, 1040, 1137, 1313, 1361, 1410, 1658, 1826, 1899, 2408, 2469, 2514, 2573, 2645, 2739, 2830, 3000, 3509], 'Indeed,': [774], 'hypometabolism': [776], 'has': [777], 'observed': [779, 4261], 'HD': [782, 813, 876, 983, 1041, 1145, 1158, 1196, 1245, 1430, 1617], '(5Feigin': [784], 'Leenders': [786], 'K.L.': [787], 'Moeller': [788], 'J.R.': [789, 1080], 'Missimer': [790], 'Kuenig': [792], 'G.': [793, 1887, 1891, 2879, 2881, 2983, 2985, 3488], 'Spetsieris': [794], 'Antonini': [796], 'Eidelberg': [798], 'Nucl.': [801], 'Med.': [802], '2001;': [803, 1651], '42:': [804], '1591-1595PubMed': [805], 'Further,': [808, 1314, 1362, 1515], 'NMR': [809], 'experiments': [810, 4004], 'symptomatic': [812], 'patients': [814], 'found': [815, 1535, 1661, 1948], 'increased': [816], 'lactate': [817], 'basal': [823], 'ganglia': [824], '(6Jenkins': [825], 'B.G.': [826, 989], 'Koroshetz': [827], 'W.J.': [828, 1082], 'Beal': [829, 920, 1024], 'M.F.': [830, 921, 985, 1025], 'Rosen': [831, 1000], 'B.R.': [832, 1001, 1076], 'Neurology.': [833], '43:': [835], '2689-2695Crossref': [836], 'Scholar),': [839, 3547], 'suggesting': [840], 'possibility': [842], 'glycolysis': [844], 'up-regulated': [846], 'compensate': [848], 'oxidative': [855, 1586], 'phosphorylation': [856, 1587], 'pathway.': [857], 'Several': [858], 'groups': [859], 'reported': [862, 1275], 'deficits': [863], 'II,': [867, 1669, 2247, 2297, 2339, 3293, 3307], 'III,': [868, 2298], 'IV': [870, 2262, 2300, 2703], 'postmortem': [872], 'brain': [873, 1618], 'tissue': [874], 'loss': [881], 'evident': [883], '(7Gu': [884], 'M.': [885, 1346, 1395, 1754, 2810], 'Gash': [886], 'M.T.': [887, 2399, 2495, 2505, 2554, 2564, 2720, 2730], 'Mann': [888], 'V.M.': [889, 933, 2395, 2497, 2501, 2556, 2560, 2712, 2726], 'Javoy-Agid': [890, 936], 'F.': [891, 937, 1096, 1106, 1164, 1174, 1294, 1300, 1450, 1460, 1801, 1811, 2718, 2722], 'Cooper': [892, 934], 'J.M.': [893, 935, 995], 'Schapira': [894, 942], 'A.H.': [895, 943, 1104, 1172, 1458, 1809], '1996;': [898, 1894], '39:': [899], '385-389Crossref': [900], '(629)': [903], '8Browne': [906], 'S.E.': [907], 'Bowling': [908], 'A.C.': [909], 'MacGarvey': [910], 'U.': [911, 1030, 2886, 2990, 3499], 'Baik': [912], 'M.J.': [913], 'Berger': [914], 'S.C.': [915], 'Muqit': [916], 'M.M.': [917], 'Bird': [918], 'E.D.': [919], '1997;': [924, 2889, 2993], '41:': [925], '646-653Crossref': [926], '(753)': [929], '9Mann': [932], 'Agid': [938], 'Y.': [939, 2457], 'Jenner': [940], 'Lancet.': [944], '1990;': [945, 2619], '336:': [946], '749Abstract': [947], '(116)': [950], 'Additionally,': [953, 1659], 'chronic': [954], 'rodents': [957], 'nonhuman': [959], 'primates': [960], 'acid': [963, 2368, 3132, 3287, 4060], '(3-NP)': [964], '(an': [965, 3703], 'irreversible': [966], 'II': [968, 2410, 2454], 'inhibitor)': [969], 'selective': [971], 'damage': [972], 'resulting': [976], 'pathology': [978], 'resembled': [982], '(10Beal': [984], 'Brouillet': [986], 'Jenkins': [988], 'Ferrante': [990, 1016], 'R.J.': [991, 1017], 'Kowall': [992, 1022], 'N.W.': [993, 1023], 'Miller': [994], 'Storey': [996], 'Srivastava': [998], 'R.': [999, 1019, 1646, 2808], 'Hyman': [1002], 'B.T.': [1003], 'Neurosci.': [1005, 1086, 1131], '13:': [1007, 1355, 1404], '4181-4192Crossref': [1008], '11Brouillet': [1012], 'Hantraye': [1014], 'Dolan': [1018], 'Leroy-Willig': [1020], 'Proc.': [1026, 2882, 2986, 3495], 'Natl.': [1027, 2883, 2987, 3496], 'Acad.': [1028, 2884, 2988, 3497], 'Sci.': [1029, 2885, 2989, 3498], 'S.': [1031, 1286, 2716, 2887, 2991, 3500], '1995;': [1033], '92:': [1034], '7105-7109Crossref': [1035], '(434)': [1038], 'models': [1043, 1427], 'extremely': [1046], 'useful': [1047, 1150], 'delineating': [1049], 'pathogenic': [1050], 'processes': [1051], '(12Sanchez': [1056], 'I.': [1057], 'Mahlke': [1058], 'C.': [1059, 3048], 'Yuan': [1060], 'Nature.': [1062], '2003;': [1063, 1306, 3052], '421:': [1064], '373-379Crossref': [1065], '(455)': [1068], '13Panov': [1071], 'A.V.': [1072], 'Gutekunst': [1073], 'C.A.': [1074], 'Leavitt': [1075], 'Hayden': [1077], 'M.R.': [1078], 'Burke': [1079], 'Strittmatter': [1081], 'Greenamyre': [1083], 'J.T.': [1084], 'Nat.': [1085], '2002;': [1087, 2638], '5:': [1088], '731-736Crossref': [1089], '(878)': [1092], '14Trettel': [1095], 'Rigamonti': [1097, 1165, 1451, 1802], 'Hilditch-Maguire': [1099, 1167, 1453, 1804], 'Wheeler': [1101, 1169, 1297, 1455, 1806], 'V.C.': [1102, 1170, 1298, 1456, 1807], 'Sharp': [1103, 1171, 1457, 1808], 'Persichetti': [1105, 1173, 1299, 1459, 1810], 'Cattaneo': [1107, 1175, 1461, 1812], 'MacDonald': [1109, 1177, 1301, 1347, 1396, 1463, 1814], 'Hum.': [1111, 1179, 1303, 1351, 1400, 1465, 1816], 'Mol.': [1112, 1180, 1304, 1352, 1401, 1466, 1817], 'Genet.': [1113, 1181, 1305, 1353, 1402, 1467, 1818], '2000;': [1114, 1182, 1468, 1819], '9:': [1115, 1183, 1469, 1820], '2799-2809Crossref': [1116, 1184, 1470, 1821], '(508)': [1119, 1187, 1473, 1824], '15Li': [1122], 'S.H.': [1123], 'Cheng': [1124], 'A.L.': [1125, 3461, 3494], 'Li': [1126, 1128], 'H.': [1127], 'X.J.': [1129], '1999;': [1132, 2818], '19:': [1133], '5159-5172Crossref': [1134], 'Cell': [1138, 1738, 1879, 3463], 'derived': [1140], 'knock-in': [1146, 1790], 'mice': [1147, 1786], 'particularly': [1149, 1687], 'tools': [1151], 'examining': [1153], 'molecular': [1155], 'pathogenesis.': [1159, 1431], 'Trettel': [1160, 1293], 'colleagues': [1162], '(14Trettel': [1163, 1449, 1800], 'knock': [1197], '(HdhQ111/Q111)': [1199], '(HdhQ7/Q7)': [1202], 'mice.': [1203], 'STHdhQ111/Q111': [1205, 1251, 1278, 1420, 1773, 3882, 3895, 4038, 4050, 4158, 4287], 'line': [1207, 1764, 1775, 2958, 4026], 'expresses': [1208], 'at': [1211, 1869, 1940, 2022, 2047, 2061, 2352, 2369, 2419, 2489, 2528, 2657, 2942, 2966, 3522], 'endogenous': [1212, 1766], 'levels,': [1213, 3072, 3914], 'therefore': [1215], 'genetically': [1218], 'accurate': [1219], 'model': [1221, 1240], 'As': [1224, 2897], 'most': [1229], 'affected': [1230], 'region': [1231], 'origin': [1236], 'makes': [1241], 'optimal': [1243, 1952, 3424, 3598, 3610], 'studies.': [1246], 'Previous': [1247], 'studies': [1248, 1415, 1989, 3585, 3605], 'using': [1249, 2034, 2531, 3038, 3129, 3336, 3351], 'STHdhQ7/Q7': [1253, 1366, 1422, 1762, 3362, 3879, 4035, 4047, 4155, 4284], 'provided': [1256], 'indirect': [1257], 'huntingtin-expressing': [1267], 'cells.': [1269, 1678, 1729, 1930, 3603, 4052], 'Reduced': [1270], 'cAMP': [1271], 'theSTHdhQ7/Q7': [1283], '(16Gines': [1285], 'Seong': [1287], 'I.S.': [1288], 'Fossale': [1289], 'Ivanova': [1291], 'Gusella': [1295], 'J.F.': [1296], '497-508Crossref': [1308], '(243)': [1311], 'sensitive': [1320, 1334, 1688, 2445, 2539], 'than': [1321, 3526, 4112], '3-NP-induced': [1326], 'toxicity,': [1327], 'however': [1328], 'equally': [1333], 'rotenone': [1336, 2152, 2234, 2294, 2435, 2541, 3223, 4014], '(a': [1337], 'I': [1339, 1616, 2516, 2549, 2582, 2598, 4319], 'inhibitor)-induced': [1340], '(17Ruan': [1343, 1392], 'Q.': [1344, 1393], 'Lesort': [1345, 1394], 'Johnson': [1349, 1398], 'G.V.': [1350, 1399], '2004;': [1354, 1403], '669-681Crossref': [1356, 1405], '(81)': [1359, 1408], '3-NP': [1369, 1391], 'resulted': [1370], 'initiation': [1373], 'apoptotic': [1375], 'death,': [1377], 'whereas': [1378], 'expressing': [1380, 1442, 1765, 1776], 'died': [1383], 'nonapoptotic': [1386], 'process': [1387], 'treated': [1389], 'Overall': [1411, 1698], 'these': [1412, 1437, 1988, 2921], 'other': [1414], 'clearly': [1416, 1580], 'studying': [1429], 'In': [1432, 2039, 2229, 3001, 3950], 'study,': [1434], 'we': [1435, 1516, 1660, 2575, 2837, 3902], '(STHdhQ111/Q111)': [1444, 1539], '(STHdhQ7/Q7)': [1447], 'examine': [1477], 'respiration.': [1488], 'A': [1489, 1907, 1937, 1967, 1981, 2111, 2314, 2687], 'O2': [1493, 1543, 2108, 2222, 2783], 'consumption,': [1494], 'delineate': [1505], 'bioenergetic': [1513], 'processes.': [1514], 'performed': [1517, 2318, 2351, 3266], 'threshold': [1518, 2331, 2796, 2910, 2975, 3003], 'analyses': [1519, 4314], 'compare': [1521], 'handling': [1522, 1567], 'inhibition': [1524, 2965], 'different': [1526, 3739, 3863, 4130], 'We': [1534], 'consumption': [1544, 2109, 2223, 2784, 3382, 4003], 'rates': [1548, 2027, 2326, 3632, 3645, 3730, 3759, 3793, 4010, 4229], '(STHdhQ7/Q7).': [1553], 'differences': [1559, 3722, 4259], 'activities.': [1564], 'Also,': [1565], 'inhibitors': [1570, 3412, 4308], 'Our': [1578], 'suggest': [1581, 1681], 'compromises': [1585], 'but': [1592], 'This': [1602, 2132, 2283, 3421, 3831, 3994, 4094, 4138], 'agreement': [1605], 'previous': [1608], 'study': [1609, 1703], 'demonstrated': [1611], 'changes': [1622], '(18Guidetti': [1632], 'Charles': [1634], 'V.': [1635], 'Chen': [1636], 'E.Y.': [1637], 'Reddy': [1638], 'P.H.': [1639], 'Kordower': [1640], 'J.H.': [1641], 'Whetsell': [1642], 'W.O.': [1644], 'Schwarcz': [1645], 'Tagle': [1647], 'D.A.': [1648], '169:': [1652], '340-350Crossref': [1653], '(167)': [1656], 'thresholds': [1665, 2833, 4313], 'I,': [1668, 2240, 2338], 'III': [1671, 2254, 2341, 2471], 'low': [1673, 3559], 'cultured': [1676, 1829, 3436], 'may': [1685], 'be': [1686, 1950, 2547, 3404, 3416, 3579], 'events': [1690], 'result': [1692, 3835], 'compromised': [1694], 'activity.': [1697], 'results': [1700], 'increase': [1704, 3248], 'understanding': [1706], 'mitochondria': [1714, 3432], 'provide': [1719], 'novel': [1720], 'important': [1722], 'data': [1723, 2803, 3012, 3348, 3930], 'regarding': [1724], 'bioenergetics': [1726], 'Materials—All': [1730], 'chemicals': [1731], 'Sigma-Aldrich': [1734], 'unless': [1735], 'otherwise': [1736], 'noted.': [1737], 'Culture—Conditionally': [1739], 'immortalized': [1740], 'progenitor': [1743], 'lines,': [1745, 3757], 'obtained': [1748, 3014], 'gift': [1751], 'Dr.': [1753], 'MacDonald,': [1755], 'study.': [1760], 'normal': [1767], 'homozygous': [1771, 1788], '111': [1780], 'glutamines': [1781], 'prepared': [1783, 3919], 'HdhQ111/Q111': [1789], 'mice,': [1791], 'respectively.': [1792], 'Both': [1793], 'described': [1798, 1884, 1996, 2312, 2601, 2709, 2805, 2841, 2898, 2980, 3043, 3924], 'previously': [1799, 1885, 1997, 2602, 2710, 2806, 2981, 3044], 'Cells': [1827], "Dulbecco's": [1831], 'modified': [1832], "Eagle's": [1833], 'medium': [1834], '(Mediatech': [1835, 1853, 1863], 'Inc.)': [1836], 'supplemented': [1837, 3200], '4%': [1839, 1845], 'fetal': [1840], 'bovine': [1841, 1846, 2101, 3168], 'serum': [1842, 1848, 2102, 3169], '(HyClone)': [1843], 'growth': [1847], '(HyClone),': [1849], '2': [1850, 2092, 2130, 2171, 2227, 2281, 2754, 2764, 3090, 3254], 'mm': [1851, 1909, 1912, 1915, 2093, 2749, 2755, 3080, 3083, 3164, 3173], 'l-glutamine': [1852], 'Inc.),': [1854], '100': [1856, 1860], 'units/ml': [1857], 'penicillin': [1858], 'μg/ml': [1861, 3161], 'streptomycin': [1862], 'Inc.).': [1864], 'grown': [1868], '33': [1870], '°C': [1871, 2063], 'humidified': [1874], 'atmosphere': [1875], 'containing': [1876, 1938], '5%': [1877], 'CO2.': [1878], 'Permeabilization—Cells': [1880], 'permeabilized': [1882, 1971, 2030, 2114, 2209, 2269, 3185, 3274, 3374, 3591, 4046], '(19Hofhaus': [1886], 'Shakeley': [1888], 'R.M.': [1889], 'Attardi': [1890, 2880, 2984], 'Methods': [1892, 2004, 2460], 'Enzymol.': [1893, 2005, 2461], '264:': [1895], '476-483Crossref': [1896], 'Briefly,': [1900, 2646], 'trypsinized,': [1903], 'resuspended': [1904, 1934, 2085, 3187], 'media': [1906, 1936, 1966, 1980, 2686, 3189], '(20': [1908, 2201, 3327], 'HEPES,': [1910], '10': [1911, 3163], 'MgCl2,': [1913], '250': [1914], 'sucrose,': [1916], 'pH': [1917, 2098, 2757, 3085, 3176], '7.3),': [1918], 'counted': [1920], 'followed': [1921, 2134, 2285, 2838, 3354], 'permeabilization': [1923], 'aliquots': [1925], '×': [1928, 2117, 2652, 2669, 2693, 3097, 4164], '107': [1929, 2118, 2670, 4165], 'An': [1931, 2069], 'aliquot': [1932], 'digitonin': [1939, 1992, 3376, 3563, 3571, 3594, 3613], 'final': [1942], 'concentration': [1943, 1953, 3018, 3569, 3599], '0.015%': [1945, 3612], '(which': [1946], 'preliminary': [1955, 3584], 'studies)': [1956], 'mixed': [1958], 'min.': [1961, 2131, 2192, 2228, 2282, 3091], 'After': [1962, 2672, 3233], 'centrifugation,': [1963], 'digitonin-containing': [1965], 'removed,': [1969], 'washed': [1974], 'large': [1977], 'volume': [1978], 'remove': [1983], 'any': [1984, 3741, 4271], 'remaining': [1985], 'digitonin.': [1986], 'For': [1987, 2193, 3271], 'commercially': [1990], 'available': [1991], 'purified': [1994], '(20Kun': [1998], 'Kirsten': [2000], 'Piper': [2002], 'W.N.': [2003], '1979;': [2006], '55:': [2007], '115-118Crossref': [2008], '(84)': [2011], 'stored': [2015], '10%': [2018], 'stock': [2019], 'Me2SO': [2021], '–20': [2023], '°C.': [2024, 2354], 'Polarographic': [2025], 'Measurements—Respiration': [2026], 'measured': [2033, 2187, 2413, 2474, 3588, 3761, 3903, 3922, 4044, 4064, 4246, 4310], 'oxygraph': [2036], '(Hansatech': [2037], 'Instruments).': [2038], 'instrument': [2041], 'Clark-type': [2043], 'electrode': [2044], 'placed': [2046, 2121], 'bottom': [2049], 'water-jacketed': [2052], 'chamber.': [2054, 2081], 'During': [2055], 'measurements,': [2056, 2196], 'chamber': [2058, 2124, 2762], 'thermostatted': [2060], '37': [2062], 'sealed': [2065], 'plunger.': [2068], 'electromagnetic': [2070], 'stirrer': [2071], 'bar': [2072], 'mix': [2076, 2207], 'contents': [2078], 'Permeabilized': [2082, 2740, 3878, 4283], 'buffer': [2089, 2747, 3078, 3152, 3193, 3199], '(media': [2090], 'A,': [2091], 'potassium': [2094], 'phosphate': [2095], '(KH2PO4:K2HPO4,': [2096], '1:1.78),': [2097], '7.1,': [2099], '1%': [2100], 'albumin)': [2103], 'immediately': [2106], 'measurements.': [2110, 3383], 'suspension': [2112, 2267], '(1': [2116, 2157, 2448], 'cells/ml)': [2119], 'allowed': [2126], 'equilibrate': [2128], 'addition': [2137, 2288, 2378, 2440, 3764], 'substrates:': [2140], 'glutamate': [2141, 2302, 3685, 3821, 3983, 4016], '(10': [2142, 2146, 2153, 2542, 3212, 3216, 3224], 'mm)': [2143, 2150, 2158, 2177, 2243, 2449, 2775, 2779, 3213, 3221], 'plus': [2144, 2151, 2159, 2215, 2293, 2303, 3214, 3222, 3686, 3701, 3822, 3852, 3984, 4013, 4017], 'malate': [2145, 2304, 3215, 3687, 3823, 3985], 'mm),': [2147, 3208, 3217], '(5': [2149, 3220], 'μm),': [2154, 3228], 'ascorbate': [2156, 2773, 3700, 3851], 'dihydrochloride': [2161], '(TMPD)': [2162], '(200': [2163, 3239], 'μm).': [2164, 2771], 'State': [2165, 2323, 3616, 3660, 3727, 3787, 3790], '4': [2166, 2658, 3082, 3617, 3728, 3788], 'monitored': [2169, 2225, 3252], 'min': [2172, 2656, 2765, 3255], 'after': [2173, 2584, 3762], 'ADP': [2175, 2310, 3238], '(1.5': [2176], 'added': [2179, 2204, 2264, 3242], 'initiate': [2181], 'state': [2182, 3624, 3838, 4008], '3': [2183, 2324, 3625, 3661, 3791, 3839, 4009], 'further': [2190, 3885, 4290], '2–4': [2191], 'uncoupled': [2194, 3770], '4-(trifluoromethoxy)phenylhydrazone': [2199], '(FCCP)': [2200], 'μm)': [2202, 2258, 2543, 3240], '(succinate': [2214, 2292], 'rotenone)': [2216, 3678, 3819, 3981], 'medium,': [2220], 'inhibitor': [2231, 2321, 3017, 3205], 'titration': [2232], 'experiments,': [2233], '(1–50': [2235], 'nm)': [2236, 2250], 'inhibit': [2238, 2245, 2252, 2260], 'malonate': [2241], '(0.05–5': [2242], 'antimycin': [2248], '(1–100': [2249], 'KCN': [2256, 2433, 2769], '(1–500': [2257, 2770], 'chamber,': [2274], 'mixture': [2277], 'incubated': [2279, 2743], 'substrates': [2291, 3210, 3745, 3828, 3991], 'I),': [2307], 'then': [2309, 3094], 'above.': [2313], 'separate': [2315], 'incubation': [2316], 'each': [2320, 2909, 4239], 'concentration.': [2322], 'analysis.': [2332, 3024], 'Enzyme': [2333, 4032], 'Assays—Citrate': [2334], 'synthase,': [2335, 4057], 'enzyme': [2342, 4041, 4152], 'determined': [2345, 2359, 2519, 2706, 2954, 3037, 3123, 3283, 3634], 'spectrophotometrically.': [2346], 'All': [2347, 3644], 'assays': [2349], '30': [2353], 'Citrate': [2355, 4075], 'synthase': [2356, 3652, 4076, 4126, 4181, 4189, 4199, 4209, 4253], 'activity': [2357, 2411, 2455, 2472, 2517, 2550, 2583, 2704, 3653, 4054, 4077, 4127, 4237, 4254, 4269], 'rate': [2362, 2444, 2523, 2538, 3772, 3960], 'color': [2364], 'change': [2365], '5,5′-dithiobis-(2-nitrobenzoic)': [2367], '412': [2370], 'nm.': [2371, 2421], 'reaction': [2373, 2423, 2746, 3192], 'initiated': [2375, 2437], 'oxaloacetate': [2380], 'acetyl-CoA': [2385], '(21Robinson': [2386], 'J.B.': [2387], 'Brent': [2388], 'L.G.': [2389], 'Sumegi': [2390], 'B.': [2391, 3046], 'Srere': [2392], 'P.A.': [2393], 'Darley-Usmar': [2394, 2496, 2500, 2555, 2559, 2725], 'Rickwood': [2396, 2502, 2561, 2727], 'Wilson': [2398, 2494, 2504, 2553, 2563, 2719, 2729], 'Mitochondria,': [2400, 2506, 2565, 2731], 'Practical': [2402, 2508, 2567, 2733], 'Approach.': [2403, 2509, 2568, 2734], 'IRL': [2404, 2510, 2569, 2735], 'Press,': [2405, 2511, 2570, 2736], 'London1987:': [2406, 2512, 2571, 2737], '160-161Google': [2407], 'Complex': [2409, 2470, 2515, 2702, 4318], 'reduction': [2416, 3832], '2,6-dichlorophenolindophenol': [2418], '600': [2420], 'succinate,': [2428], 'ubiquinone-2.': [2442], '2-thenyltrifluoroacetone': [2447], 'taken': [2451, 2545], '(22Hatefi': [2456], 'Stiggall': [2458], 'D.L.': [2459, 3459], '1978;': [2462], '53:': [2463], '21-27Crossref': [2464], '(170)': [2467], 'monitoring': [2476], 'oxidation': [2478, 2525], 'ubiquinone-2': [2481, 2532], 'cytochrome': [2483, 4195], 'c': [2484, 4196, 4205], 'acceptor': [2488], '550': [2490], 'nm': [2491, 2530], '(23Ragan': [2492, 2551], 'C.I.': [2493, 2552], 'Lowe': [2498, 2557], 'P.N.': [2499, 2558], '79-112Google': [2513, 2572], 'measuring': [2521], 'NADH': [2527], '340': [2529], 'acceptor.': [2536], 'Because': [2574], 'able': [2578], 'detect': [2580], 'freeze-thaw': [2585], 'treatment,': [2586, 2675], 'osmotic': [2587, 2673], 'shock': [2588, 2674], 'sonication': [2590], 'prepare': [2594], 'samples': [2595, 2648, 2676, 3145, 3276], 'assay': [2599, 3041, 3133, 3155, 3196], 'modifications': [2604], '(24Chretien': [2605], 'Bourgeron': [2607], 'T.': [2608, 2814], 'Rotig': [2609], 'Munnich': [2611], 'Rustin': [2613], 'Biochem.': [2615], 'Biophys.': [2616, 2636, 3538], 'Commun.': [2618], '173:': [2620], '26-33Crossref': [2621], '(55)': [2624], '25Helmerhorst': [2627], 'Murphy': [2629], 'M.P.': [2630], 'Troxler': [2631], 'R.F.': [2632], 'Oppenheim': [2633], 'F.G.': [2634], 'Biochim.': [2635, 3537], 'Acta.': [2637, 3539], '1556:': [2639], '73-80Crossref': [2640], '(67)': [2643], 'thawed': [2647], 'centrifuged': [2650, 2678, 3095], '(16,000': [2651], 'g': [2653, 3098], '5': [2655, 3301], '°C),': [2659], 'pellet': [2662, 2682], 'diluted': [2664], 'cold': [2666], 'water': [2667], '(2–4': [2668], 'cells/ml).': [2671], 'again,': [2679], 'dissolved': [2684], 'sonicated': [2689], 'ice': [2691], '(3': [2692], '20': [2694, 3315], 's)': [2695], 'prior': [2696], 'use': [2698], 'assay.': [2701, 3109, 3150, 3288], 'polarographically': [2707], '(26Darley-Usmar': [2711], 'Capaldi': [2713], 'R.A.': [2714], 'Takamiya': [2715], 'Millett': [2717], 'Malatesta': [2721], 'Sarti': [2723], '113-152Google': [2738], '(50': [2748], '0.3%': [2751], 'Tween': [2752], '80,': [2753], '7.5)': [2758], 'without': [2766], 'Subsequently,': [2772], '(3.5': [2774], 'TMPD': [2777, 3702, 3853], '(0.35': [2778], 'added,': [2781], 'observed.': [2786], 'Threshold': [2787, 2951, 4280], 'Curves': [2788], 'Determination': [2790], 'Thresholds': [2792], 'Spare': [2794, 2972], 'Capacities—The': [2795], 'curves': [2797, 3004], 'constructed': [2799], 'raw': [2802], '(27Rossignol': [2807], 'Malgat': [2809], 'Mazat': [2811], 'J.P.': [2812], 'Letellier': [2813], 'Biol.': [2816, 3447, 3464], 'Chem.': [2817, 3448], '274:': [2819], '33426-33432Abstract': [2820], '(238)': [2828], 'spare': [2835, 2931], 'capacities,': [2836], 'method': [2840], 'Refs.': [2843], '28Mallajosyula': [2844], 'J.K.': [2845, 2847], 'Andersen': [2846], 'Nicholls': [2848], 'D.G.': [2849], 'On-line': [2850], 'Abstract': [2851], 'Viewer': [2852], 'Itinerary': [2854], 'Planner,': [2855], '34th': [2856], 'Annual': [2857], 'Meeting': [2858], 'Society': [2861, 2870], 'Neuroscience,': [2863, 2872], 'San': [2864], 'Diego,': [2865], 'California,': [2866], 'October': [2867], '23–27,': [2868], '2004.': [2869], 'Washington,': [2873], 'C.2004Google': [2875], 'Scholar': [2876], '29Villani': [2878], '94:': [2890, 2994], '1166-1171Crossref': [2891, 2995], '(176)': [2894, 2998], 'Scholar.': [2896], 'previously,': [2899], 'least-square': [2901], 'regression': [2902, 2957, 3023], 'beyond': [2904], 'inflection': [2906, 3006], 'point': [2907, 3007], 'curve': [2911], 'extrapolated': [2913], 'zero': [2915], 'inhibition.': [2917, 2950], 'intersection': [2919], 'ordinate': [2925], 'axis': [2926], 'capacity': [2932, 2973, 3752], 'subtracting': [2937], '100%': [2938], 'value': [2941, 2976], 'unaffected': [2946, 2970], 'values': [2952], 'equations,': [2959], 'percentage': [2962], '(100%).': [2971], 'terms': [2977], '(29Villani': [2982], 'several': [3002], 'evident;': [3010], 'therefore,': [3011], 'points': [3013], 'every': [3016], 'linear': [3022], 'Analysis': [3025], 'Levels': [3028, 3872], 'Production—ATP': [3031], 'luciferin/luciferase': [3040, 3108], '(30Drew': [3045], 'Leeuwenburgh': [3047], 'Am.': [3049], 'Physiol.': [3051], '285:': [3053], 'R1259-R1267Crossref': [3054], '(128)': [3057], 'utilizing': [3060], 'Turner': [3062], 'Designs': [3063], 'TD': [3064], '20/20': [3065], '(Turner': [3066], 'Designs)': [3067], 'luminometer.': [3068], 'collected': [3075], 'lysis': [3077], '(100': [3079], 'Tris,': [3081], 'EDTA,': [3084], '7.75)': [3086], 'boiled': [3088], 'Samples': [3092], '(1000': [3096], '1min),': [3100], 'supernatants': [3103], 'normalized': [3113, 4066, 4086, 4120, 4248], 'content': [3116, 3140, 4111], 'samples.': [3119], 'Protein': [3120], 'concentrations': [3121, 3281, 3561], 'lysates': [3126, 3917], 'before': [3127], 'boiling': [3128], 'bicinchoninic': [3131, 3286], '(Pierce)': [3134], 'calculate': [3138, 3797], 'amount': [3143], 'Reaction': [3151], 'contained': [3156], '70': [3157], 'μm': [3158], 'luciferin,': [3159], '0.05': [3160], 'luciferase,': [3162], 'magnesium': [3165], 'acetate,': [3166, 3175], '0.063%': [3167], 'albumin,': [3170], '150': [3172], 'Tris': [3174], '7.5.': [3177], 'adenylate': [3203], 'kinase': [3204], '(di(adenosine)pentaphosphate,': [3206], '0.15': [3207], '(glutamate': [3211], 'μm)),': [3225], 'luciferin': [3226], '(70': [3227], 'luciferase': [3230], '(0.05': [3231], 'μg/ml).': [3232], 'initial': [3235], 'luminescence': [3236, 3250], 'reading,': [3237], 'reaction,': [3245], 'kinetic': [3258], 'mode.': [3259], 'Western': [3260], 'Blot': [3261], 'Analysis—Western': [3262], 'blot': [3263], 'according': [3267], 'general': [3269], 'protocols.': [3270], 'analysis,': [3273], 'sonicated,': [3278], 'Antibodies': [3289], 'were:': [3291], '70-kDa': [3295], 'subunit': [3296, 3310], 'monoclonal': [3297, 3311, 3324], 'antibody': [3298, 3312, 3325], '(Molecular': [3299, 3313], 'Probes,': [3300, 3314], 'μg': [3302, 3316, 3328], 'protein,': [3304, 3318, 3330], '1:10,000),': [3305], '30-kDa': [3309], '1:1,000),': [3319], 'voltage-dependent': [3321], 'anion': [3322], 'channel': [3323], '(Calbiochem)': [3326], '1:2,000).': [3331], 'Statistical': [3332], 'Analysis—Results': [3333], 'analyzed': [3335, 3350], "Student's": [3337, 3356], 't': [3338, 3342, 3357], 'test': [3339, 3343], 'paired': [3341], 'indicated.': [3345], "Chauvenet's": [3352], 'Criterion': [3353], 'test.': [3358], 'Respiratory': [3359, 3798], 'Properties': [3360], '(Wild-type)': [3363, 3880, 4036, 4285], 'STH-dhQ111/Q111': [3365, 3370], '(Mutant)': [3366, 3883, 4039, 4288], 'Cells—STHdhQ7/Q7': [3367], '(mutant)': [3371, 3896, 4051, 4159], '(0.015%)': [3377], 'oxygen': [3381, 4002], 'Digitonin': [3384], 'binds': [3385], 'cholesterol': [3387, 3511], 'eukaryotic': [3390], 'plasma': [3391, 3529, 3556], 'membrane': [3392, 3530, 3557], 'creates': [3394], 'pores': [3395], 'soluble': [3398], 'components': [3399], 'cytosol': [3402], 'can': [3403, 3414], 'released': [3405], 'substrates,': [3409], 'cofactors,': [3410], 'easily': [3415], 'introduced': [3417], 'cell.': [3420], 'approach': [3422], 'maintenance': [3427], 'coupled,': [3429], 'metabolically': [3430], 'active': [3431], 'isolated': [3434], '(31Harris': [3438], 'S.I.': [3439], 'Balaban': [3440], 'R.S.': [3441, 3476], 'Barrett': [3442], 'L.': [3443], 'Mandel': [3444], 'L.J.': [3445], '1981;': [3449], '256:': [3450], '10319-10328Abstract': [3451], '32Granger': [3458], 'Lehninger': [3460, 3493], '95:': [3466], '527-535Crossref': [3467], '(320)': [3470], '33Dubinsky': [3473], 'W.P.': [3474], 'Cockrell': [3475], 'FEBS': [3477], 'Lett.': [3478], '1975;': [3479], '59:': [3480], '39-43Crossref': [3481], '(38)': [3484], '34Fiskum': [3487], 'Craig': [3489], 'S.W.': [3490], 'Decker': [3491], 'G.L.': [3492], '1980;': [3502], '77:': [3503], '3430-3434Crossref': [3504], '(223)': [3507], 'Although': [3510], 'present': [3514], 'outer': [3517], 'membrane,': [3519], 'substantially': [3523], 'lower': [3524, 3775, 3967, 4021], '(35Colbeau': [3531], 'Nachbaur': [3533], 'Vignais': [3535], 'P.M.': [3536], '1971;': [3540], '249:': [3541], '462-492Crossref': [3542], '(510)': [3545], 'thus': [3548], 'making': [3549], 'possible': [3551], 'selectively': [3553], 'permeabilize': [3554], 'if': [3558, 3567], 'enough': [3560], 'used.': [3565, 3615, 3747], 'high': [3574], 'enough,': [3575], 'will': [3578], 'inadequately': [3580], 'permeabilized.': [3581], 'Therefore': [3582], '0.005–0.02%': [3593], 'revealed': [3606, 3957], '(in': [3618, 3626], 'absence': [3620], 'ADP)': [3622, 3630], 'between': [3637, 3723, 3864, 3943, 4131, 4262], '(Fig.': [3642, 3785, 3829, 3869, 3948, 3992, 4005], '1A).': [3643, 3786], 'expressed': [3647], 'per': [3648, 4143], 'unit': [3649], 'citrate': [3651, 4056, 4125, 4180, 4188, 4198, 4208, 4252], 'normalize': [3655], 'content.': [3659, 4074], '(with': [3677, 3818, 3980, 4011], '(feeds': [3679], 'electrons': [3680, 3689, 3709], 'II)': [3683], '(feed': [3688], 'I)': [3692], 'substrates.': [3697], 'IV)': [3712], 'substrate,': [3718, 3858], 'observe': [3749], 'maximal': [3750], 'uncoupler': [3767], 'FCCP.': [3768], 'Control': [3799], 'Ratios': [3800], '(RCRs).': [3801], 'RCRs': [3802, 3860], '1B).': [3830, 3870], 'shown': [3846], 'Fig.': [3848], '1A.': [3849], 'When': [3850, 4119], 'Production': [3876], 'Cells—To': [3884, 4289], 'analyze': [3886, 4291], 'energetic': [3888], 'status': [3889], 'STH-dhQ7/Q7': [3892], 'cells,': [3897, 4235], 'especially': [3898], 'mitochondria,': [3901], 'total': [3904, 3915, 3940], 'production.': [3910], 'under': [3925], '“Materials': [3926], 'Methods.”': [3928], 'difference': [3937], '2A).': [3949], 'contrast,': [3951], 'measurement': [3952], '2B).': [3993], 'consistent': [3996], '1A)': [4006], 'where': [4007], 'malate)': [4018], 'wild-type.': [4030], 'Activities': [4033], 'Cells—Mitochondrial': [4040], 'tricarboxylic': [4059], 'cycle': [4061], 'enzyme,': [4062], 'number': [4069, 4089], 'decreased': [4079], '(25%)': [4084], '(Table': [4092, 4136, 4255], 'I).': [4093, 4137, 4256], 'corresponded': [4095], 'observation': [4098], 'appear': [4102], 'smaller': [4103], 'size': [4105], '∼25%': [4108], 'less': [4109], '(data': [4116], 'shown).': [4118], 'content,': [4124], 'suggests': [4139], 'load': [4142], 'mass': [4145], 'lines.Table': [4150], 'IMitochondrial': [4151], 'cellsEnzymeUnitsWild-typeMutantmean': [4160], '±': [4161, 4167, 4169, 4175, 4177, 4183, 4185, 4191, 4193, 4201, 4203, 4211, 4213], 'S.E.Citrate': [4162], 'synthasenmol/min/1': [4163], 'cells545.18': [4166], '25.96407.62': [4168], '20.2ap': [4170], '<': [4171, 4216], '0.001.Citrate': [4172], 'synthasenmol/min/mg': [4173], 'protein467.29': [4174], '29.14463.64': [4176], '30.07NADH': [4178], 'dehydrogenasenmol/min/unit': [4179, 4187], 'activity35.06': [4182], '4.2235.54': [4184], '1.98Succinate': [4186], 'activity8.862': [4190], '0.668.278': [4192], '1.54Ubiquinone': [4194], 'oxidoreductasenmol/min/unit': [4197], 'activity77.84': [4200], '11.7754.61': [4202], '10.44Cytochrome': [4204], 'oxidasenmol': [4206], 'O2/min/unit': [4207], 'activity159.39': [4210], '10.89182.51': [4212], '22.65a': [4214], 'p': [4215], '0.001.': [4217], 'Open': [4218], 'table': [4219], 'new': [4222], 'tab': [4223], 'understand': [4225], 'why': [4226], 'four': [4242, 4274], 'units': [4250], 'No': [4257], 'Inhibitor': [4277], 'Titrations': [4278], 'Analyses': [4281], 'sensitivity': [4300], 'out.': [4317], 'wa': [4320]}, 'cited_by_api_url': 'https://api.openalex.org/works?filter=cites:W2047298360', 'counts_by_year': [{'year': 2024, 'cited_by_count': 11}, {'year': 2023, 'cited_by_count': 5}, {'year': 2022, 'cited_by_count': 9}, {'year': 2021, 'cited_by_count': 8}, {'year': 2020, 'cited_by_count': 7}, {'year': 2019, 'cited_by_count': 8}, {'year': 2018, 'cited_by_count': 14}, {'year': 2017, 'cited_by_count': 12}, {'year': 2016, 'cited_by_count': 13}, {'year': 2015, 'cited_by_count': 12}, {'year': 2014, 'cited_by_count': 10}, {'year': 2013, 'cited_by_count': 15}, {'year': 2012, 'cited_by_count': 22}], 'updated_date': '2025-01-07T23:28:26.632341', 'created_date': '2016-06-24'}