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3550, 3585, 3589, 3610, 3614, 3620, 3624, 3638, 3645, 3649, 3658, 3669, 3681, 3711, 3719, 3729, 3734, 3749, 3777, 3781, 3784, 3795, 3798, 3810, 3813, 3825, 3843, 3875, 3886, 3890, 3905, 3918, 3927, 3956, 3965, 3981, 3988, 4005, 4012], 'npc1': [16, 212, 561, 1666], 'gene.': [17, 213], 'These': [18, 129, 214, 325, 540, 1205, 1236, 1902, 2370, 3291], 'studies': [19, 215, 1206, 1237, 2371, 2651, 3149, 3301, 3862], 'evaluated': [20, 216], 'effect': [22, 218, 1007, 2779, 3069, 3225, 3656, 3789], 'of': [23, 27, 32, 44, 73, 78, 91, 97, 107, 111, 126, 137, 162, 177, 219, 223, 228, 240, 269, 274, 287, 293, 303, 307, 322, 333, 358, 373, 403, 413, 416, 437, 494, 549, 586, 629, 657, 698, 700, 725, 867, 879, 951, 954, 989, 1003, 1008, 1021, 1034, 1050, 1058, 1068, 1080, 1083, 1103, 1152, 1160, 1165, 1180, 1185, 1188, 1202, 1216, 1232, 1243, 1249, 1271, 1299, 1342, 1365, 1374, 1468, 1519, 1524, 1558, 1697, 1733, 1748, 1760, 1782, 1799, 1811, 1854, 1865, 1871, 1873, 1879, 1889, 1897, 1908, 1933, 2078, 2091, 2118, 2142, 2187, 2214, 2278, 2342, 2410, 2423, 2464, 2499, 2589, 2594, 2609, 2616, 2628, 2635, 2643, 2677, 2681, 2701, 2714, 2755, 2768, 2785, 2808, 2864, 2870, 2940, 2951, 2957, 2998, 3039, 3045, 3054, 3061, 3097, 3104, 3115, 3133, 3141, 3188, 3212, 3218, 3254, 3280, 3320, 3332, 3352, 3382, 3404, 3411, 3423, 3440, 3456, 3465, 3470, 3472, 3506, 3515, 3531, 3549, 3578, 3588, 3606, 3616, 3623, 3640, 3651, 3662, 3668, 3671, 3718, 3733, 3742, 3752, 3783, 3794, 3797, 3812, 3822, 3889, 3893, 3915, 3926, 3930, 3955], 'genetic': [24, 54, 220, 250, 617, 653, 991, 1090, 1170, 1222, 1330, 2327, 2533, 2671, 3216, 3244, 3257, 3339, 3490, 3887], 'background,': [25, 152, 221, 348, 654, 2241], 'deletion': [26, 77, 222, 273, 1184, 2863, 2939], 'additional': [28, 224, 1189, 3068], 'genes,': [29, 225, 1190], 'and': [30, 70, 122, 158, 160, 226, 266, 318, 354, 356, 420, 491, 649, 667, 677, 692, 763, 799, 887, 942, 992, 1048, 1191, 1356, 1429, 1476, 1521, 1537, 1569, 1602, 1622, 1628, 1715, 1745, 1762, 1839, 1959, 1983, 1989, 2047, 2052, 2136, 2211, 2290, 2301, 2339, 2357, 2366, 2378, 2386, 2442, 2450, 2453, 2472, 2483, 2546, 2693, 2737, 2740, 2878, 2893, 2898, 3334, 3355, 3362, 3396, 3414, 3604, 3700, 3715, 3762, 3787, 3827, 3837, 3900, 3941, 3949, 3974, 3979, 3987, 4011], 'administration': [31, 106, 161, 227, 302, 357, 2956, 3796, 3811], 'several': [33, 229, 1194, 3294], 'agents': [34, 163, 230, 359, 1195], 'on': [35, 231, 1225, 1256, 1649, 1820, 2238, 2539, 2780, 3180, 3226, 3657], 'age': [37, 67, 134, 233, 263, 330, 662, 1065, 1119, 1135, 1229, 1761, 1872, 2336, 2560, 2610, 2617, 2698, 2782, 3046, 3105, 3112, 3228, 3271, 3304, 3373, 3442, 3481, 3546, 3659, 3730, 3847, 3865], 'at': [38, 68, 135, 234, 264, 331, 1066, 1120, 1136, 1230, 1362, 1730, 1757, 1862, 1868, 1930, 2204, 2337, 2561, 2606, 2637, 2699, 2783, 3042, 3101, 3113, 3229, 3272, 3305, 3374, 3443, 3482, 3547, 3660, 3731, 3819, 3848, 3866], 'death': [39, 69, 136, 235, 265, 332, 681, 802, 834, 1067, 1137, 1231, 2014, 2338, 2562, 2638, 2700, 2784, 2836, 3114, 3230, 3273, 3306, 3375, 3483, 3526, 3548, 3661, 3732, 3849, 3867], 'murine': [42, 238, 547, 836, 973, 2016, 2838], 'model': [43, 239, 548, 585, 974, 1298, 1696], 'this': [45, 241, 550, 647, 857, 1004, 1093, 1127, 1203, 1221, 1392, 1783, 1800, 2119, 2137, 2678, 2952, 2970, 3064, 3158, 3528, 3788], 'disorder.': [46, 242, 1005], 'Such': [47, 243], 'factors': [48, 244, 1161, 3903], 'as': [49, 165, 245, 361, 522, 555, 979, 1025, 1075, 1572, 1574, 1942, 2149, 2313, 2460, 2732, 2734, 3342, 3815, 3868], 'differing': [50, 246], 'strain': [51, 151, 247, 347, 1178, 1618, 2572, 2623, 3340], 'background': [52, 59, 248, 255, 1179, 1259, 2573, 2624, 3217, 3258, 3341, 3888], 'or': [53, 82, 105, 114, 182, 249, 278, 301, 310, 378, 1777, 1791, 1894, 2130, 2435, 2503, 2794, 3173, 3199, 3251, 3385, 3454, 3491, 3648], 'drift': [55, 251, 1171, 2328, 3245], 'within': [56, 252, 870], 'given': [58, 254, 3041], 'npc1−/−': [62, 93, 99, 139, 258, 289, 295, 335, 1123, 1167, 1376, 1611, 2242, 2348, 2379, 2424, 2556, 2600, 2629, 2665, 2685, 2716, 2787, 3056, 3085, 3117, 3289, 3314, 3508, 3551, 3735], 'mouse': [63, 140, 259, 336, 1124, 3170, 3194, 3261, 3552], 'significantly': [64, 119, 143, 260, 315, 339, 2249, 2764, 2960, 3156, 3184, 3266, 3433, 3487], 'altered': [65, 261, 1477, 3486], 'degree': [72, 268], 'organ': [74, 270, 516], 'disease.': [75, 271, 1094, 1154, 1204], 'Genetic': [76, 272], 'Siat9': [79, 275, 2762], '(GM3': [80, 276], 'synthetase)': [81, 277], 'Nr1h2': [83, 279], '[liver': [84, 280], 'X': [85, 281, 397, 1493, 3004], 'receptor': [86, 282, 395, 398, 1426, 1494, 2953, 2971, 3674], '(LXR)β]': [87, 283], 'shortened': [88, 284, 2943], 'life': [90, 286, 2962, 3051, 3621, 3782], 'animals.': [94, 128, 290, 324, 1235, 2325, 3345, 3405], 'Daily': [95, 291], 'treatment': [96, 292, 1192, 2155, 3091], 'mice': [100, 296, 622, 644, 695, 726, 770, 804, 910, 1335, 1377, 1428, 1561, 1646, 1716, 1754, 1855, 2093, 2099, 2122, 2216, 2279, 2349, 2362, 2380, 2411, 2425, 2505, 2557, 2578, 2717, 2771, 3086, 3220, 3315, 3424, 3736, 3916], 'with': [101, 113, 297, 309, 623, 650, 661, 696, 727, 767, 771, 805, 831, 911, 1143, 1193, 1336, 1403, 1806, 1885, 2011, 2178, 2322, 2350, 2668, 2833, 3092, 3192, 3297], 'an': [102, 298, 864, 980, 996, 1792, 1807, 2641, 2662, 2669, 3075], 'LXR': [103, 166, 299, 362, 1793, 3705], 'agonist': [104, 300, 1794, 2959], 'single': [109, 305, 1859, 2416, 2691, 3037, 3817], 'dose': [110, 306, 1810, 3038, 3818], 'cyclodextrin,': [112, 308], 'without': [115, 311], 'neurosteroid': [117, 313], 'allopregnanolone,': [118, 314], 'slowed': [120, 316], 'neurodegeneration': [121, 317, 768], 'increased': [123, 319, 660, 865, 2311, 2806, 2867, 3094], 'lifespan': [125, 321, 1164, 2580, 2767, 2944, 3187], 'these': [127, 178, 323, 374, 1015, 1059, 1069, 1634, 1642, 2330, 2347, 2407, 2529, 2598, 2682, 2786, 2936, 3020, 3055, 3098, 3148, 3210, 3368, 3457, 3473, 3663, 3833, 3857], 'data': [130, 326, 2500], 'illustrate': [131, 327], 'that': [132, 328, 426, 431, 518, 552, 1086, 1099, 1196, 2402, 3136, 3150, 3165, 3350, 3431, 3479, 3494, 3840, 3860, 3863, 3879], 'can': [141, 337, 1011, 1116, 3155, 3221, 3265, 3484, 3880], 'be': [142, 338, 1012, 1073, 3122, 3357, 3485, 3792, 3852], 'influenced': [144, 340], 'many': [146, 342, 1060, 1100, 3151], 'factors,': [147, 343], 'including': [148, 344, 1169], 'differences': [149, 345, 2201, 2394], 'other': [153, 349, 1852, 3260, 3476, 3901], 'inactivating': [154, 350], 'gene': [155, 351, 562, 1434, 2763, 3238, 3533], 'mutations': [156, 352], '(Siat9': [157, 353], 'lxrβ),': [159, 355], 'such': [164, 360, 1009, 1024], 'agonists': [167, 363], 'and,': [168, 364, 531, 3907], 'particularly,': [169, 365], 'cyclodextrin.': [170, 366], 'It': [171, 367], 'currently': [173, 369], 'not': [174, 370, 1107, 3200, 3205, 3268, 3583], 'clear': [175, 371, 3146], 'which': [176, 183, 372, 379, 984, 1104, 1121, 3303, 3317, 3535, 3608], 'effects': [179, 375, 988, 1082, 2538, 3021, 3211], 'nonspecific': [181, 377, 3153], 'might': [184, 380, 1087, 3137], 'relate': [185, 381, 1108, 3496], 'directly': [186, 382, 1109], 'to': [187, 383, 445, 537, 985, 998, 1072, 1110, 1140, 1145, 1219, 1594, 1721, 1814, 1841, 1926, 1961, 1979, 1993, 2101, 2114, 2127, 2166, 2563, 2581, 2695, 2813, 2875, 2925, 2935, 3063, 3121, 3127, 3177, 3202, 3209, 3299, 3390, 3462, 3497, 3520, 3676, 3745, 3791, 3851, 3884, 3896, 3911, 3921, 3977], 'molecular': [189, 385, 1147, 3826], 'defect': [190, 386, 1091, 1112, 1223, 3501], 'present': [191, 387], 'NPC1': [194, 390, 430, 955, 1253, 3237, 3720], 'syndrome.': [195, 391], 'low': [392, 1404, 1423, 1767], 'density': [393, 1405, 1424], 'lipoprotein': [394, 1406, 1425, 1576], 'liver': [396, 527, 672, 800, 832, 906, 1026, 1492, 1995, 2012, 2295, 2344, 2358, 2456, 2468, 2815, 2834, 3003], 'University': [402, 1364], 'Texas': [404, 1366], 'Southwestern': [405, 1367], 'one': [412, 1973, 2228, 3453], 'number': [415, 1020, 1159, 1390, 1907, 3081, 3096], 'lysosomal': [417], 'storage': [418, 618, 1331, 3581], 'diseases': [419], 'results': [421, 2146, 3878], 'from': [423, 440, 900, 1262, 1267, 1380, 1384, 1395, 2219, 2227, 2244, 2257, 2363, 2382, 2404, 2428, 2501, 2549, 2575, 3147, 3259, 3328, 3434, 3710, 3882, 3964, 4001], 'inactivates': [427], 'protein': [429, 916, 1254, 3611], 'responsible': [433, 3537, 3612], 'for': [434, 646, 875, 1041, 2084, 2153, 2230, 2674, 3163, 3538, 3613, 3842, 3944, 3952], 'movement': [436], 'unesterified': [438, 827, 2007, 2735, 2829], 'cholesterol': [439, 506, 593, 658, 828, 868, 898, 1114, 1306, 1525, 1577, 1704, 1768, 1788, 1984, 2008, 2053, 2291, 2355, 2465, 2736, 2793, 2809, 2830, 2871, 2899, 2927, 2999, 3027, 3642, 3678, 3708, 3747, 3838], 'late': [442], 'endosomal/lysosomal': [443], 'compartment': [444, 873], 'cytosol': [447], 'every': [449, 665, 2656, 3420], 'cell': [450, 673, 680, 801, 833, 1053, 2013, 2835, 3080, 3763], '(1.Patterson': [451], 'M.C.': [452], 'Vanier': [453, 3571], 'M.T.': [454, 3572], 'Suzuki': [455, 3573], 'K.': [456, 578, 1291, 1464, 1509, 1547, 1689, 2721, 3574, 3755], 'Morris': [457, 565, 1278, 1676], 'J.A.': [458, 566, 790, 934, 1279, 1677, 2041, 2887], 'Carstea': [459, 567, 1280, 1678], 'E.': [460], 'Neufeld': [461, 3563], 'E.B.': [462, 3564], 'Blanchette-Mackie': [463], 'E.J.': [464], 'Pentchev': [465, 686, 3569], 'P.G.': [466, 605, 687, 1318, 3570], 'C:': [470], 'lipid': [472, 1567, 3760], 'trafficking': [473], 'disorder.in:': [474], 'Scriver': [475], 'C.R.': [476], 'Beaudet': [477], 'A.L.': [478], 'Sly': [479], 'W.S.': [480], 'Valle': [481], 'D.': [482, 3695], 'Childs': [483], 'B.': [484, 488, 821, 1511, 2001, 2037, 2823, 2883], 'Kinzler': [485], 'K.W.': [486], 'Vogelstein': [487], 'The': [489, 1006, 1665, 2087, 2421, 3932], 'Metabolic': [490], 'Molecular': [492], 'Bases': [493], 'Inherited': [495], 'Disease.': [496], 'McGraw': [497], 'Hill,': [498], 'New': [499], 'York2001:': [500], '3611-3633Google': [501], 'Scholar).': [502, 642, 855, 971, 1971, 2073, 2919], 'As': [503, 2390], 'result,': [505], 'accumulates': [507], 'virtually': [509], 'all': [510, 1632, 2168, 2197, 2680, 3471], 'tissues': [511], 'body,': [514], 'causing': [515], 'dysfunction': [517, 535], 'may': [519, 1197], 'manifest': [520], 'clinically': [521], 'hepatosplenomegaly,': [523], 'prolonged': [524, 2579, 2961, 3048], 'neonatal': [525, 670], 'jaundice,': [526], 'dysfunction,': [528, 676], 'pulmonary': [529, 675, 1029, 2880], 'failure,': [530], 'ultimately,': [532], 'progressive': [533], 'neurological': [534, 1046], 'secondary': [536], 'selective': [538, 678], 'neurodegeneration.': [539], 'clinical': [541, 1045, 2089, 3282, 3586], 'findings': [542], 'are': [543, 946, 1623, 2147, 2513, 3370], 'reproduced': [544], 'also': [553, 1401, 2111, 2492, 2535, 2709, 3459, 3997], 'arose': [554], 'spontaneous': [557], '(2.Loftus': [563, 1276, 1674], 'S.K.': [564, 1277, 1675], 'E.D.': [568, 1281, 1679], 'Gu': [569, 1282, 1680], 'J.Z.': [570, 1283, 1681], 'Cummings': [571, 1284, 1682], 'C.': [572, 683, 712, 892, 932, 1285, 1683, 2039, 2885, 3689], 'Brown': [573, 1286, 1411, 1684], 'A.': [574, 1287, 1503, 1685, 3692], 'Ellison': [575, 1288, 1686], 'J.': [576, 613, 704, 1289, 1326, 1420, 1505, 1579, 1687, 3684, 3701, 3722], 'Ohno': [577, 1290, 1688], 'Rosenfeld': [579, 1292, 1690], 'M.A.': [580, 752, 1293, 1691, 2980], 'Tagle': [581, 1294, 1692], 'D.A.': [582, 1295, 1693, 2994], 'et': [583, 1296, 1694], 'al.Murine': [584, 1297, 1695], 'disease:': [589, 1302, 1700], 'homeostasis': [594, 1307, 1705, 3000], 'gene.Science.': [595, 1308, 1706], '1997;': [596, 1309, 1707], '277:': [597, 1310, 1708], '232-235Crossref': [598, 1311, 1709], 'PubMed': [599, 640, 742, 782, 813, 851, 928, 967, 1312, 1353, 1441, 1487, 1532, 1589, 1710, 1847, 1967, 2031, 2069, 2749, 2853, 2915, 3015, 3599, 3772], 'Scopus': [600, 743, 783, 814, 852, 968, 1313, 1442, 1488, 1533, 1590, 1711, 1848, 1968, 2032, 2070, 2750, 2854, 2916, 3016, 3600, 3773], '(700)': [601, 1314, 1712], 'Google': [602, 641, 709, 745, 785, 816, 854, 929, 970, 1315, 1354, 1444, 1490, 1535, 1592, 1713, 1850, 1970, 2034, 2072, 2752, 2856, 2918, 3018, 3602, 3775], 'Scholar,': [603, 710, 746, 786, 817, 930, 1316, 2035], '3.Pentchev': [604, 1317], 'Boothe': [606, 1319], 'A.D.': [607, 1320], 'Kruth': [608, 1321], 'H.S.': [609, 1322], 'Weintroub': [610, 1323], 'H.': [611, 748, 1324, 1456, 3686], 'Stivers': [612, 1325], 'Brady': [614, 1327], 'R.O.': [615, 1328], 'A': [616, 1329, 1371], 'BALB/c': [621, 652, 1258, 1334, 2240, 2352, 2555], 'metabolic': [625, 1338, 2396], 'block': [626, 858, 1339], 'esterification': [628, 1341], 'exogenous': [630, 1343], 'cholesterol.J.': [631, 1344], 'Biol.': [632, 1345], 'Chem.': [633, 1346], '1984;': [634, 1347], '259:': [635, 1348], '5784-5791Abstract': [636, 1349], 'Full': [637, 846, 848, 923, 925, 962, 964, 1350, 1584, 1586, 2026, 2028, 2064, 2066, 2848, 2850, 2910, 2912], 'Text': [638, 847, 849, 924, 926, 963, 965, 1351, 1585, 1587, 2027, 2029, 2065, 2067, 2849, 2851, 2911, 2913], 'PDF': [639, 850, 927, 966, 1352, 1588, 2030, 2068, 2852, 2914], 'In': [643, 1605, 1630, 1914, 1972, 2074, 2196, 2649, 2774, 2802, 2933, 3207, 3724], 'homozygous': [645], 'mutation,': [648], 'concentration': [656, 1932, 1985], 'becomes': [659], 'nearly': [664, 1631, 3106], 'organ,': [666], 'there': [668, 2391, 2776, 3073], 'cholestasis,': [671], 'death,': [674], 'nerve': [679, 1052], '(4.Xie': [682], 'Turley': [684, 715, 755, 793, 824, 893, 935, 1499, 2004, 2042, 2826, 2888], 'S.D.': [685, 716, 756, 794, 825, 894, 936, 1500, 2005, 2043, 2827, 2889], 'Dietschy': [688, 717, 759, 795, 822, 895, 937, 1514, 2002, 2044, 2824, 2890], 'J.M.': [689, 718, 760, 796, 823, 896, 938, 1458, 1515, 2003, 2045, 2825, 2891], 'Cholesterol': [690, 719, 797, 939], 'balance': [691], 'metabolism': [693, 3839], 'loss': [697, 1557, 3709], 'function': [699, 1027, 1187, 1996, 2359, 2881, 2942], 'protein.Am.': [703], 'Physiol.': [705], '1999;': [706], '276:': [707], 'E336-E344PubMed': [708], '5.Xie': [711], 'Burns': [713], 'D.K.': [714], 'sequestered': [721], 'brains': [724, 2713], 'but': [732, 2611, 3058], 'turnover': [733], 'increased.J.': [735], 'Neuropathol.': [736, 776, 2743], 'Exp.': [737, 777, 2744], 'Neurol.': [738, 778, 2745], '2000;': [739, 920, 1529, 1581, 3596], '59:': [740], '1106-1117Crossref': [741], '(97)': [744], '6.Li': [747], 'Repa': [749], 'J.J.': [750, 1498], 'Valasek': [751], 'Beltroy': [753], 'E.P.': [754, 788, 819, 1999, 2821], 'German': [757], 'D.C.': [758], 'Molecular,': [761], 'anatomical,': [762], 'biochemical': [764], 'events': [765, 1148], 'associated': [766], 'disease.J.': [775, 840, 2020, 2058, 2842, 2904], '2005;': [779, 810, 1484], '64:': [780], '323-333Crossref': [781], '(102)': [784], '7.Beltroy': [787], 'Richardson': [789, 933, 2040, 2886], 'Horton': [791], 'J.D.': [792], 'accumulation': [798, 2054, 2900, 3514], 'disease.Hepatology.': [809], '42:': [811], '886-893Crossref': [812], '(104)': [815], '8.Beltroy': [818], 'Liu': [820, 2000, 2822], 'Lysosomal': [826, 2006, 2828], 'content': [829, 2009, 2831], 'correlates': [830, 2010, 2832], 'Lipid': [841, 918, 957, 2021, 2059, 2843, 2905], 'Res.': [842, 919, 958, 2022, 2060, 2844, 2906], '2007;': [843, 2023, 2061, 2845, 2907], '48:': [844, 2024, 2062, 2846, 2908], '869-881Abstract': [845, 2025, 2847], '(57)': [853, 2033, 2855], 'Despite': [856, 1055], 'intracellular': [860], 'sterol': [861, 882], 'movement,': [862], 'however,': [863, 2922], 'rate': [866], 'synthesis': [869, 1470, 2758, 3615], 'cytoplasmic': [872], 'allows': [874], 'essentially': [876], 'normal': [877, 908, 947], 'rates': [878], 'plasma': [880, 1990, 2306, 2490, 3750], 'membrane': [881, 3751], 'turnover,': [883], 'bile': [884], 'acid': [885], 'synthesis,': [886], 'steroid': [888, 943], 'hormone': [889, 944], 'production': [890], '(9.Xie': [891], 'Centripetal': [897], 'flow': [899], 'extrahepatic': [902], 'organs': [903], 'through': [904], 'mutated': [912], '(NPC1).J.': [917], '41:': [921], '1278-1289Abstract': [922], '10.Xie': [931], 'substrate': [940], 'pools': [941], 'levels': [945, 1040, 2308, 2488], 'face': [950], 'mutational': [952], 'inactivation': [953, 3530, 3605], 'protein.J.': [956], '2006;': [959], '47:': [960], '953-963Abstract': [961], '(40)': [969], 'This': [972, 1155, 3801, 3958], 'has': [975, 3426, 3517, 3654], 'proved': [976], 'very': [977], 'valuable': [978], 'experimental': [981, 1084, 1234, 1736, 2657, 3363, 3397, 3412, 3919], 'animal': [982, 1360, 1805, 2447, 3118, 3176, 3190, 3213], 'explore': [986], 'various': [990, 1217, 1635, 2707, 3134], 'pharmacological': [993, 3492], 'manipulations': [994, 1010, 1085, 3135, 3154, 3493], 'attempt': [997], 'better': [999], 'understand': [1000], 'pathophysiology': [1002, 1151], 'assessed': [1013], 'animals': [1016, 1070, 1250, 1264, 1539, 1724, 1976, 2243, 2401, 2530, 2552, 2604, 2630, 2686, 2788, 3333, 3437, 3626, 3664], 'evaluating': [1018, 3130], 'end': [1022, 1062, 3125, 3159, 3309, 3871], 'points,': [1023, 1063], 'tests,': [1028], 'diffusion': [1030], 'capacity,': [1031], 'level': [1033, 3650], 'macrophage': [1035, 2051, 2897], 'infiltration': [1036], 'tissues,': [1038], 'mRNA': [1039], 'different': [1042, 1987, 2511, 2515, 2532, 3489, 3891, 3898], 'inflammatory': [1043], 'proteins,': [1044], 'function,': [1047], 'quantitation': [1049], 'specific': [1051], 'numbers.': [1054], 'availability': [1057], 'precise': [1061], 'continues': [1071], 'used': [1074, 1978, 2165], 'relatively': [1077, 3401], 'easy': [1078], 'measure': [1079, 1980, 1994, 3925], 'alter': [1088, 1117, 1198, 2926, 3157, 3746], 'However,': [1095, 2274, 2673, 3467, 3856], 'we': [1096], 'recently': [1097], 'found': [1098, 3502], 'manipulations,': [1101], 'some': [1102, 1917, 2603, 2647, 3276], 'probably': [1105], 'do': [1106, 3495], 'transport,': [1115], 'dies.': [1125], 'If': [1126], 'case,': [1130, 1607], 'then': [1131], 'changes': [1132, 1175, 1226, 3025, 3834, 3845], 'could': [1138, 3022, 3460], 'lead': [1139, 3461], 'erroneous': [1141], 'conclusions': [1142], 'respect': [1144, 3298], 'dictating': [1149], 'NPC': [1153, 2215, 3142], 'report': [1156], 'outlines': [1157], 'affecting': [1162, 3904], 'mouse,': [1168], 'colony,': [1174, 2406], 'mutant': [1182, 2770, 3625, 3785], 'animals,': [1183, 3894, 3906], 'natural': [1200], 'history': [1201], 'suggest': [1207, 3478], 'potential': [1209, 3474, 3876], 'hazards': [1210], 'inherent': [1211], 'interpreting': [1213], 'relevance': [1215], 'treatments': [1218], 'overcoming': [1220], 'based': [1224], 'were': [1238, 1400, 1620, 1647, 1738, 1755, 1856, 1977, 2082, 2110, 2123, 2133, 2202, 2217, 2236, 2309, 2372, 2392, 2412, 2426, 2458, 2461, 2491, 2687, 2791], 'undertaken': [1239], 'using': [1240], 'eight': [1241, 2502], 'groups': [1242, 1853, 1910, 2277, 2409, 2422, 3398, 3892, 3920], 'genetically': [1244], 'modified': [1245], 'mice.': [1246], 'One': [1247], 'group': [1248, 1373, 2417, 2627, 2667, 2692, 3422, 3741], 'lacking': [1251], 'functional': [1252], '(npc1−/−)': [1255], 'was': [1260, 1378, 1668, 1924, 1991, 2094, 2138, 2164, 2183, 2194, 2631, 2659, 2777, 2798, 3074, 3960], 'derived': [1261, 1379, 1621, 2218, 2381, 2427, 2574], 'heterozygous': [1263, 1381], 'originally': [1265], 'obtained': [1266, 1992], 'National': [1269], 'Institutes': [1270], 'Health': [1272, 3969], '9': [1273], 'years': [1274], 'ago': [1275], 'Scholar)': [1355, 1593], 'maintained': [1357, 1648, 2237], 'colony': [1361, 1399, 2247, 2260, 3248, 3331], '(UTSW)': [1368], 'Medical': [1369, 1751, 3991, 4008], 'School.': [1370, 1752], 'second': [1372, 2861], 'similar': [1375, 2281], 'founders': [1382], 'purchased': [1383], 'Jackson': [1386, 2212, 2259, 2367, 2387, 2436], 'Laboratories': [1387, 2437], '(BALB/cNctr-Npc1m1N/J;': [1388], 'stock': [1389], '003092)': [1391], 'year.': [1393], 'Animals': [1394], 'original': [1397], 'UTSW': [1398, 1750, 2210, 2246, 2365, 2385, 2431], 'crossed': [1402], 'receptor-deficient': [1407], '(ldlr−/−)': [1408], '(11.Ishibashi': [1409], 'S.': [1410, 1551, 3694, 3967], 'M.S.': [1412], 'Goldstein': [1413], 'J.L.': [1414, 1460, 2982], 'Gerard': [1415], 'R.D.': [1416], 'Hammer': [1417], 'R.E.': [1418], 'Herz': [1419], 'Hypercholesterolemia': [1421], 'knockout': [1427], 'its': [1430], 'reversal': [1431], 'adenovirus-mediated': [1433], 'delivery.J.': [1435], 'Clin.': [1436], 'Invest.': [1437], '1993;': [1438], '92:': [1439], '883-893Crossref': [1440], '(1273)': [1443], 'Scholar),': [1445, 1491, 1536, 1714, 1851, 2753, 2857, 3019, 3603, 3726, 3776], 'Siat9−/−': [1446], '(12.Yamashita': [1447], 'T.': [1448, 3688], 'Wu': [1449, 1544, 3559], 'Y-P.': [1450, 3560], 'Sandhoff': [1451, 1463], 'R.': [1452, 1462, 3562], 'Werth': [1453], 'N.': [1454], 'Mizukami': [1455], 'Ellis': [1457], 'Dupree': [1459], 'Geyer': [1461], 'Proia': [1465, 3575], 'R.L.': [1466, 3576], 'Interruption': [1467], 'ganglioside': [1469, 2757, 3580], 'produces': [1471], 'central': [1472, 3008, 3031], 'nervous': [1473, 3009, 3032], 'system': [1474], 'degeneration': [1475], 'axon-glial': [1478], 'interactions.Proc.': [1479], 'Natl.': [1480], 'Acad.': [1481], 'Sci.': [1482], 'USA.': [1483], '102:': [1485], '2725-2730Crossref': [1486], '(197)': [1489], 'β-deficient': [1495], '(lxrβ−/−)': [1496], '(13.Repa': [1497], 'Lobaccaro': [1501], 'J-M.': [1502], 'Medina': [1504], 'Li': [1506], 'L.': [1507, 1829, 1949], 'Lustig': [1508], 'Shan': [1510], 'Heyman': [1512], 'R.A.': [1513], 'Mangelsdorf': [1516], 'D.J.': [1517], 'Regulation': [1518, 2997], 'absorption': [1520], 'ABC1-mediated': [1522], 'efflux': [1523], 'RXR': [1527], 'heterodimers.Science.': [1528], '289:': [1530], '1524-1529Crossref': [1531], '(1150)': [1534], 'abca1−/−': [1538, 2577], '(14.Christiansen-Weber': [1540], 'T.A.': [1541], 'Voland': [1542], 'J.R.': [1543], 'Y.': [1545, 3558], 'Ngo': [1546], 'Roland': [1548], 'B.L.': [1549, 3996], 'Nguyen': [1550], 'Peterson': [1552], 'P.A.': [1553], 'Fung-Leung': [1554], 'W-P.': [1555], 'Functional': [1556], 'ABCA1': [1559], 'causes': [1562], 'severe': [1563], 'placental': [1564], 'malformation,': [1565], 'aberrant': [1566, 1718], 'distribution,': [1568], 'kidney': [1570], 'glomerulonephritis': [1571], 'well': [1573, 2733], 'high-density': [1575], 'deficiency.Am.': [1578], 'Pathol.': [1580], '157:': [1582], '1017-1029Abstract': [1583], '(206)': [1591], 'yield': [1595], 'double': [1596], 'knockouts': [1597], 'designated': [1598, 1624], 'npc1−/−/ldlr−/−,': [1599], 'npc1−/−/Siat9−/−,': [1600], 'npc1−/−/lxrβ−/−,': [1601], 'npc1−/−/abca1−/−,': [1603], 'respectively.': [1604], 'each': [1606, 1804, 2154], 'appropriate': [1609], 'control': [1610, 2666, 2684, 3313, 3361, 3395, 3885, 3897], 'littermates': [1612], 'having': [1613], 'same': [1615, 1784, 2221, 2269, 3330, 3338], 'respective,': [1616], 'mixed': [1617, 2571], 'backgrounds': [1619, 2534, 2548], 'npc1−/−/ldlr+/+,': [1625], 'npc1−/−/Siat9+/+,': [1626], 'npc1−/−/lxrβ+/+,': [1627], 'npc1−/−/abca1+/+.': [1629], 'cases,': [1633, 2198, 3277], 'crosses': [1636], 'occurred': [1637], 'over': [1638, 2640, 3379], '>10': [1639], 'generations.': [1640], 'After': [1641], 'crosses,': [1643], 'female': [1645], 'low-cholesterol': [1651], '(0.03%,': [1652], 'w/w)': [1653, 1770, 1790], 'rodent': [1654, 1771], 'diet': [1655, 1772, 1785, 1802], '(No.': [1656, 1773], '7002;': [1657], 'Harlan': [1658, 1775], 'Teklad,': [1659], 'Madison,': [1660], 'WI)': [1661], 'during': [1662, 2267], 'pregnancies.': [1664], 'genotype': [1667, 3417], 'determined': [1669], 'PCR': [1672], 'method': [1673], 'exhibiting': [1717], 'lifespans': [1719], 'relative': [1720, 2454], 'similarly': [1722], 'treated': [1723, 3344], '(outliers)': [1725], 'had': [1726, 2224, 2280, 2536, 3066], 'their': [1727, 3945], 'genotypes': [1728], 'confirmed': [1729], 'time': [1732, 2270, 3929], 'death.': [1734, 2143, 3444, 3931], 'All': [1735, 1753, 2144], 'protocols': [1737], 'approved': [1739], 'Institutional': [1742], 'Animal': [1743], 'Care': [1744], 'Use': [1746], 'Committee': [1747], 'weaned': [1756], '19': [1758], 'days': [1759, 1870, 2585, 2608, 2615, 2645, 2703, 3044, 3103, 3384, 3821], 'fed': [1763, 2792], 'ad': [1764], 'libitum': [1765], '(0.02%,': [1769], '7001;': [1774], 'Teklad)': [1776], 'ground': [1779, 3171], 'meal': [1780], 'form': [1781, 2117], 'containing': [1786], 'either': [1787, 1886, 2405, 2429], '(1%,': [1789], '(T0901317;': [1795], 'Cayman': [1796], 'Chemical).': [1797], 'Intake': [1798], 'latter': [1801, 2937, 3802], 'provided': [1803, 2112], 'approximate': [1808], 'daily': [1809, 3178], 'T0901317': [1812], 'equal': [1813], '50': [1815], 'mg/kg': [1816, 1882, 1939], 'body': [1817, 1883, 1940, 2282, 2446], 'weight.': [1818], 'Based': [1819], 'previous': [1822], 'publication': [1823], '(15.Griffin': [1824, 1944], 'L.D.': [1825, 1945], 'Gong': [1826, 1946], 'W.': [1827, 1947], 'Verot': [1828, 1948], 'Mellon': [1830, 1950], 'S.H.': [1831, 1951], 'involves': [1836, 1956], 'disrupted': [1837, 1957], 'neurosteroidogenesis': [1838, 1958], 'responds': [1840, 1960], 'allopregnanolone.Nat.': [1842, 1962], 'Med.': [1843, 1963], '2004;': [1844, 1964, 3769], '10:': [1845, 1965], '704-711Crossref': [1846, 1966], '(318)': [1849, 1969], 'administered': [1857], 'subcutaneous': [1860], 'injection': [1861], 'scruff': [1864], 'neck': [1867], '7': [1869, 3043, 3820], '20%': [1875], '(in': [1876], 'saline)': [1877], 'solution': [1878], '2-hydroxypropyl-β-cyclodextrin': [1880], '(4,000': [1881], 'weight)': [1884, 1941], '5.6': [1887], 'degrees': [1888, 1896], 'substitution': [1890, 1898], '(Aldrich;': [1891], 'product': [1892, 1900, 1922], '332607)': [1893], '4.5': [1895], '(Sigma;': [1899, 1921], 'H107).': [1901], 'values': [1903, 2595], 'indicate': [1904], 'average': [1906, 3050], 'hydroxypropyl': [1909], 'per': [1911, 2506], 'cyclodextrin': [1912, 1928, 3040, 3093, 3814], 'molecule.': [1913], 'addition,': [1915], 'experiments,': [1918], 'allopregnanolone': [1919, 3062], '(5α-pregnan-3α-ol-20-one)': [1920], 'P8887)': [1923], 'added': [1925], 'solutions': [1929], '1.5': [1934], 'mg/ml': [1935], '(to': [1936, 3985, 3994], 'provide': [1937], '25': [1938], 'described': [1943], 'experiment,': [1974, 2076], '56-day-old': [1975, 2376], 'weight': [1982], 'organs,': [1988], 'tests': [1997, 2360], '(8.Beltroy': [1998, 2820], '16.Liu': [2036], 'Xie': [2038, 2884], 'Receptor-mediated': [2046, 2892], 'bulk-phase': [2048, 2894], 'endocytosis': [2049, 2895], 'cause': [2050, 2896], '1710-1723Abstract': [2063, 2909], '(37)': [2071, 2917], 'another': [2075, 2229, 3740], 'samples': [2077], 'anterior': [2080], 'cerebellum': [2081], 'taken': [2083], 'histological': [2085], 'examination.': [2086], 'general': [2088], 'condition': [2090], 'monitored': [2095], 'daily.': [2096], 'Once': [2097], 'began': [2100], 'show': [2102], 'difficulty': [2103], 'accessing': [2104], 'pelleted': [2106], 'basal': [2107], 'diet,': [2108, 3354], 'they': [2109, 2132, 2223, 2790], 'access': [2113], 'powdered': [2116], 'diet.': [2120], 'When': [2121], 'no': [2124, 2393, 2778, 3067, 3655], 'longer': [2125, 2250], 'able': [2126], 'take': [2128], 'food': [2129, 3172, 3198], 'water,': [2131], 'humanely': [2134], 'euthanized,': [2135], 'considered': [2139], 'day': [2141], 'numeric': [2145], 'expressed': [2148, 2973], 'means': [2150, 2496], '±': [2151, 2252, 2262, 2497, 2565, 2583, 2946, 2964], 'SEM': [2152, 2498], 'group.': [2156, 2507], 'GraphPad': [2157], 'Prism': [2158], 'software': [2159], '(GraphPad,': [2160], 'San': [2161], 'Diego': [2162], 'CA)': [2163], 'perform': [2167], 'statistical': [2169], 'analyses.': [2170], 'To': [2171], 'compare': [2172], 'multiple': [2173], 'groups,': [2174, 2190], 'one-way': [2176], 'ANOVA': [2177], 'Neumann-Keuls': [2179], 'post': [2180], 'hoc': [2181], 'comparison': [2182, 2186], 'performed.': [2184], 'For': [2185, 2541, 3242, 3510], 'only': [2188, 2310, 3196, 3269], 'two': [2189, 2331, 2408, 3455], "Student's": [2192], 't-test': [2193], 'used.': [2195], 'statistically': [2199, 2514], 'significant': [2200, 2537], 'declared': [2203], 'P': [2205], '<': [2206, 2517], '0.05.': [2207], 'Although': [2208, 2706, 3072, 3110], 'colonies': [2213, 2235, 2332], 'source,': [2222], 'been': [2225, 3518], 'isolated': [2226], '∼9': [2231], 'years.': [2232], 'Whereas': [2233], 'both': [2234, 2276, 2334, 2383], 'lived': [2248], '(89': [2251], '1.4': [2253], 'days)': [2254, 2264, 2966, 3053, 3556, 3632], 'than': [2255], 'those': [2256], '(80': [2261], '0.8': [2263], 'when': [2265, 2621, 2789, 2795, 3367, 3415], 'observed': [2266, 3844], 'period': [2271], '(Fig.': [2272, 2284, 2288, 2296, 2299, 2303, 2315, 2586, 2618, 2704, 2772, 2800, 2967, 3070, 3087, 3108, 3249, 3263, 3284, 3387, 3633, 3665, 3737], '1A).': [2273], 'although': [2275, 3512, 3636], 'weights': [2283, 2448, 2457], '2A),': [2285], 'enlarged': [2286], 'livers': [2287], '2C),': [2289], 'concentrations': [2292, 2356, 2463], '2D),': [2297], 'spleen': [2298, 2470], '2E),': [2300], 'lung': [2302], '2F),': [2304], 'transaminase': [2307], 'half': [2312], 'much': [2314], '2H,': [2316], 'I)': [2317, 2487], 'Jackson,': [2320], 'compared': [2321, 3191], 'UTSW,': [2324], 'Thus,': [2326, 3389], 'affected': [2333], 'severity': [2341, 3279], 'background.Fig.': [2353], '2.Tissue': [2354], 'Laboratory': [2368, 2388], 'colonies.': [2369, 2389], 'carried': [2373, 2810, 2872, 3450], 'out': [2374, 3451], 'npc1+/+': [2377, 2400], 'parameters': [2397], 'came': [2403], 'combined': [2413, 2688], 'into': [2414, 2531, 2553, 2689, 3644], '(n': [2418, 2432, 2438], '=': [2419, 2433, 2439], '9).': [2420], '8)': [2434, 2440], 'stocks': [2441], 'studied': [2443], 'independently.': [2444], 'Whole': [2445], '(A)': [2449], 'absolute': [2451], '(B)': [2452], '(C)': [2455], 'measured,': [2459], '(D),': [2469], '(E)': [2471], 'lungs': [2473, 2877], '(F).': [2474], 'Alkaline': [2475], 'phosphatase': [2476], '(AP;': [2477], 'G),': [2478], 'alanine': [2479], 'aminotransferase': [2480, 2485], '(ALT;': [2481], 'H),': [2482], 'aspartate': [2484], '(AST;': [2486], 'measured.': [2493], 'Values': [2494], 'depict': [2495], 'nine': [2504], 'Bars': [2508], 'denoted': [2509], 'letters': [2512], '(P': [2516], '0.05).View': [2518], 'Large': [2519], 'Image': [2520], 'Figure': [2521], 'ViewerDownload': [2522], 'Hi-res': [2523], 'image': [2524], 'Download': [2525], '(PPT)': [2526], 'Importantly,': [2527], 'crossing': [2528], 'lifespan.': [2540], 'example,': [2542, 3164, 3243, 3511], 'introducing': [2543], 'C57BL/6': [2545], '129/SvJ': [2547], 'ldlr−/−': [2551], 'decreased': [2558, 2765], '79': [2564, 3555], '1.0': [2566], 'days,': [2567], 'whereas': [2568, 2858, 2949], 'similar,': [2570], '91': [2582], '1.1': [2584], '1B).': [2587], 'Also': [2588], 'note': [2590], 'range': [2593, 2634, 3381], 'seen': [2596, 3286], '142': [2599, 2683], 'mice,': [2601, 3057, 3786], 'where': [2602], 'died': [2605], '63': [2607], 'others': [2612], 'reached': [2613], '109': [2614], '1).': [2619, 3388], 'Even': [2620], 'constant,': [2632], 'ages': [2636], 'varied': [2639], 'interval': [2642], '20': [2644, 3383], 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[3033], 'More': [3034], 'strikingly,': [3035], 'markedly': [3047, 3779], '(>108': [3052], 'addition': [3060, 3208], 'regimen': [3065], '3E).': [3071], '∼80%': [3076], 'reduction': [3077], 'Purkinje': [3079], '4A': [3088], ',': [3089], 'B),': [3090], 'cells': [3099, 3505, 3753], 'surviving': [3100], '49': [3102], '3-fold': [3107], '4C).': [3109], 'would': [3119], 'seem': [3120], 'definitive': [3124], 'point': [3126, 3872], 'use': [3128, 3864, 3912], 'effectiveness': [3132, 3319], 'elucidate': [3138], 'pathogenesis': [3140], 'disease,': [3143], 'it': [3144, 3347, 3808], 'presumably': [3152, 3239], 'point.': [3160, 3310], 'We': [3161], 'found,': [3162], 'offering': [3166], 'partially': [3168], 'debilitated': [3169], 'subjecting': [3174], 'exercise': [3179, 3203, 3356], 'rotarod': [3182], 'apparatus': [3183], 'prolongs': [3185], 'offered': [3195], 'solid': [3197], 'subjected': [3201], '(data': [3204], 'shown).': [3206], 'husbandry,': [3214, 3353], 'have': [3222, 3336, 3400], 'major': 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