Publication Information

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'https://openalex.org/W2221323276', 'https://openalex.org/W2137644470', 'https://openalex.org/W2073761443', 'https://openalex.org/W1509019340', 'https://openalex.org/W104571512'], 'abstract_inverted_index': {'Defects': [0, 253], 'in': [1, 15, 86, 187, 236, 242, 254, 268, 339, 440, 489, 495, 584, 658, 670, 699, 795, 800, 876, 884, 909, 933, 1095, 1106, 1189, 1237, 1258, 1284, 1402, 1411, 1422, 1432, 1554, 1587, 1618, 1777, 1798, 1807, 1850, 2041, 2056, 2111, 2147, 2165, 2341, 2455, 2606, 2690, 2792, 2800, 2830, 2914, 2953, 2977, 2998, 3058, 3066, 3128, 3217, 3440, 3459, 3532, 3544, 3595, 3606, 3626, 3657, 3676, 3683, 3805, 3908, 4189, 4237], 'Niemann-Pick,': [2, 255, 575, 822, 825], 'Type': [3, 256, 576, 595, 597, 823, 826], 'C-1': [4, 257], 'protein': [5, 158, 258, 411, 847, 1444, 1470, 1480, 1502, 1558, 1625, 1802, 2153, 2268, 2358, 3283, 3372, 3656, 3927, 3959, 4209, 4236, 4288, 4323], '(NPC1)': [6, 259], 'cause': [7, 260], 'cholesterol,': [8, 168, 261, 421, 724, 782, 952, 2175], 'sphingolipids,': [9, 262], 'phospholipids,': [10, 263], 'and': [11, 20, 48, 66, 104, 127, 173, 204, 264, 273, 301, 319, 357, 380, 426, 457, 623, 643, 681, 685, 694, 720, 726, 731, 961, 989, 1027, 1089, 1150, 1236, 1252, 1405, 1426, 1489, 1617, 1716, 1809, 2000, 2045, 2181, 2239, 2373, 2383, 2417, 2422, 2441, 2499, 2516, 2529, 2555, 2573, 2589, 2655, 2671, 2699, 2789, 2798, 2809, 2817, 3039, 3046, 3064, 3073, 3114, 3123, 3158, 3199, 3278, 3351, 3355, 3363, 3370, 3430, 3435, 3472, 3547, 3708, 3735, 3761, 3773, 3795, 3876, 4138, 4303], 'glycolipids': [12, 265], 'to': [13, 74, 180, 206, 216, 266, 327, 433, 459, 469, 925, 940, 964, 1118, 1153, 1254, 1503, 1546, 1721, 1894, 2163, 2253, 2261, 2271, 2344, 2683, 2740, 2963, 3009, 3020, 3141, 3188, 3305, 3330, 3411, 3417, 3637, 3697, 3930, 4211, 4283], 'accumulate': [14, 267, 722, 780], 'lysosomes': [16, 269, 941, 1262, 1406, 1504], 'of': [17, 30, 57, 118, 135, 143, 190, 202, 270, 283, 310, 371, 388, 396, 443, 455, 508, 513, 520, 586, 646, 679, 729, 741, 751, 769, 773, 798, 873, 878, 912, 950, 993, 1111, 1197, 1242, 1286, 1407, 1415, 1540, 1559, 1790, 1852, 2004, 2067, 2174, 2249, 2359, 2395, 2399, 2576, 2595, 2678, 2686, 2697, 2714, 2736, 2743, 2757, 2787, 2805, 2868, 2874, 2878, 2882, 2891, 2903, 2946, 3037, 3043, 3109, 3112, 3118, 3178, 3229, 3242, 3272, 3289, 3428, 3433, 3444, 3447, 3468, 3475, 3501, 3524, 3598, 3621, 3630, 3654, 3662, 3665, 3670, 3738, 3756, 3770, 3798, 3841, 3862, 3869, 3871, 3904, 3914, 3919, 4207, 4270, 4285, 4294, 4311, 4319], 'liver,': [18, 271], 'spleen,': [19, 272, 682], 'brain.': [21, 274], 'In': [22, 275, 1080, 1683, 2274, 2331, 3612], 'cultured': [23, 87, 276, 340, 885, 1243, 1408], 'fibroblasts,': [24, 237, 277, 490], 'NPC1': [25, 38, 63, 234, 246, 278, 291, 316, 487, 499, 590, 774, 1416, 1439, 1542, 1860, 2044, 2145, 2250, 2282, 2343, 2583, 2691, 2788, 2904, 2915, 2952, 3045, 3054, 3122, 3434, 3476, 4190], 'deficiency': [26, 160, 247, 279, 413, 500, 1845], 'causes': [27, 161, 280, 414], 'lysosomal': [28, 281, 1853, 4141, 4223], 'retention': [29, 282], 'lipoprotein-derived': [31, 284], 'cholesterol': [32, 65, 130, 214, 285, 318, 383, 467, 599, 742, 799, 829, 913, 957, 974, 981, 1005, 1032, 1039, 1100, 1105, 1116, 1259, 1632, 1723, 1782, 1854, 1896, 2008, 2061, 2284, 2290, 2374, 3874], 'after': [33, 286, 3232], 'uptake': [34, 287, 932], 'by': [35, 123, 150, 288, 376, 403, 581, 689, 792, 923, 1297, 1812, 2366, 2586, 2643, 2664, 2694, 2709, 2726, 3071, 3308, 3376, 3414, 3549, 3783, 3878, 4328], 'receptor-mediated': [36, 289, 920], 'endocytosis.': [37, 290], 'contains': [39, 292, 2389, 2489, 2506, 2522, 2535, 4221], '1278': [40, 293], 'amino': [41, 294, 2361, 2959, 4080, 4205, 4218, 4301], 'acids': [42, 295, 2362, 3971, 4064, 4070, 4081, 4136, 4150, 4206, 4302], 'that': [43, 52, 62, 296, 305, 315, 566, 676, 777, 914, 979, 1003, 1037, 1249, 1438, 1494, 1550, 1627, 1803, 2050, 2059, 2154, 2244, 2263, 2279, 2363, 2386, 3338, 3505, 3956, 4191, 4280, 4284], 'form': [44, 297], '13': [45, 298, 1446, 3932], 'membrane-spanning': [46, 299, 1447], 'helices': [47, 300, 1448, 3935, 4078], 'three': [49, 302, 1485], 'large': [50, 303, 1486], 'loops': [51, 304], 'project': [53, 306], 'into': [54, 307, 1483, 3873, 3960], 'the': [55, 71, 210, 223, 308, 324, 463, 476, 570, 796, 870, 881, 910, 917, 943, 948, 959, 968, 973, 986, 1090, 1098, 1291, 1412, 1420, 1427, 1479, 1501, 1560, 1588, 1689, 1778, 1847, 2051, 2065, 2112, 2170, 2247, 2255, 2295, 2332, 2337, 2390, 2396, 2456, 2596, 2607, 2652, 2656, 2668, 2672, 2687, 2715, 2744, 2758, 2831, 2869, 2884, 2896, 3002, 3010, 3016, 3031, 3137, 3154, 3184, 3347, 3378, 3383, 3425, 3490, 3510, 3529, 3589, 3596, 3599, 3618, 3638, 3651, 3658, 3693, 3716, 3796, 3806, 3839, 3858, 3915, 3958, 3961, 4146, 4187, 4198, 4208, 4215, 4222, 4234, 4238, 4286, 4317], 'lumen': [56, 309], 'lysosomes.': [58, 311, 1107], 'We': [59, 152, 312, 405, 2257, 2378], 'showed': [60, 313, 2278], 'earlier': [61, 314], 'binds': [64, 317, 1781, 2155, 2371, 4192], 'oxysterols.': [67, 171, 320, 424], 'Here': [68, 321], 'we': [69, 322, 2143, 2277, 2335, 2351, 4194], 'localize': [70, 323, 2336, 4186], 'binding': [72, 113, 134, 179, 201, 225, 325, 366, 387, 432, 454, 478, 924, 2291, 2339, 2381], 'site': [73, 114, 226, 326, 367, 479, 2340], 'luminal': [75, 89, 188, 228, 328, 342, 441, 481, 1487, 2345, 2353, 2369, 2387, 4061, 4067, 4133], 'loop-1,': [76, 329], 'a': [77, 94, 156, 162, 197, 330, 347, 409, 415, 450, 673, 727, 738, 748, 770, 789, 793, 906, 1109, 1195, 1240, 1256, 1390, 1441, 1472, 1490, 1496, 1547, 1556, 1620, 1624, 1629, 1796, 1800, 2001, 2057, 2148, 2151, 2264, 2356, 2590, 3226, 3310, 3334, 3441, 3495, 3627, 3853, 3912, 3922, 3966, 4073, 4140, 4212], '240-amino': [78, 331], 'acid': [79, 332, 863, 1392, 2960, 3924], 'domain': [80, 333, 1691, 3917, 4076], 'with': [81, 110, 334, 363, 510, 660, 672, 1199, 1445, 1792, 1843, 1858, 2006, 2375, 2866, 3001, 3225, 3238, 3269, 3286, 3313, 3341, 3498, 3509, 3521, 3540, 3586, 3650, 3723, 3753, 3849, 4277], '18': [82, 335, 3364], 'cysteines.': [83, 336], 'When': [84, 337, 2161], 'produced': [85, 338, 2693], 'cells,': [88, 341, 1239, 2912], 'loop-1': [90, 189, 229, 343, 442, 482, 2354, 2370, 2388, 4062], 'was': [91, 121, 148, 344, 374, 401, 1295, 1551, 1687, 2298, 2602, 2639, 2660, 2681, 2720, 2738, 2864, 2996, 3018, 3139, 3156, 3166, 3169, 3186, 3211, 3236, 3251, 3266, 3284, 3303, 3327, 3339, 3374, 3492, 3516, 3538, 3633, 3695, 3847, 3882], 'secreted': [92, 345, 1811, 2365], 'as': [93, 346, 574, 1042, 1103, 1398, 1855, 2158, 2285, 2287, 2355, 2453, 2604, 2828, 2906, 3061, 3077, 3131, 3470, 3552, 3687, 3803, 3884, 4231, 4326], 'soluble': [95, 157, 348, 410, 1801, 2357], 'dimer.': [96, 116, 349, 369], 'This': [97, 350, 1387, 2600, 3168], 'loop': [98, 351], 'bound': [99, 167, 352, 420, 2007, 2283, 4275], '[3H]cholesterol': [100, 144, 178, 203, 353, 397, 431, 456, 3455], '(Kd,': [101, 354], '130': [102, 355], 'nm)': [103, 109, 356, 362], '[3H]25-hydroxycholesterol': [105, 358], '(25-HC,': [106, 359], 'Kd,': [107, 360], '10': [108, 361, 2566], 'one': [111, 364, 585, 2867, 3842], 'sterol': [112, 195, 224, 365, 448, 477, 844, 994, 2338, 3602], 'per': [115, 368, 3249], 'Binding': [117, 142, 370, 395, 3906], 'both': [119, 372], 'sterols': [120, 373, 1785], 'competed': [122, 131, 176, 375, 384, 429], 'oxysterols': [124, 377, 2156, 2168, 2234, 2372], '(24-,': [125, 378], '25-,': [126, 379], '27-HC).': [128, 381], 'Unlabeled': [129, 382], 'strongly': [132, 385, 2048], 'for': [133, 139, 177, 194, 233, 386, 392, 430, 447, 486, 1040, 1419, 1481, 1622, 2150, 2901, 3035, 3171, 3175, 3193, 3422, 3482, 3518, 3528, 3644, 3747, 3758, 3768, 3785, 3792, 3838, 4233], '[3H]cholesterol,': [136, 389], 'but': [137, 145, 169, 182, 238, 390, 398, 422, 435, 491, 745, 783, 1097, 2064, 2289], 'weakly': [138, 391], '[3H]25-HC': [140, 147, 205, 393, 400, 458, 3451, 4276], 'binding.': [141, 394], 'not': [146, 170, 183, 231, 399, 423, 436, 484, 736, 2071, 2236, 2393, 4290], 'inhibited': [149, 402], 'detergents.': [151, 404], 'also': [153, 406], 'studied': [154, 407], 'NPC2,': [155, 181, 408, 434, 1799, 3436], 'whose': [159, 412], 'similar': [163, 416, 1186, 4282], 'disease': [164, 417, 572, 775, 1288, 1794], 'phenotype.': [165, 418], 'NPC2': [166, 419, 624, 1844, 1890, 2005, 2046, 2252, 2706, 2790, 3115, 3124, 3478], 'Epicholesterol': [172, 425], 'cholesteryl': [174, 427, 944, 1044, 1091, 2411], 'sulfate': [175, 428, 2009, 2412], 'NPC1.': [184, 208, 437, 461, 2273, 2400], 'Glutamine': [185, 438], '79': [186, 439], 'NPC-1': [191, 444], 'is': [192, 230, 445, 483, 569, 579, 665, 747, 786, 887, 938, 962, 1430, 1440, 1786, 1804, 2241, 2364, 3928, 4304], 'important': [193, 446], 'binding;': [196, 449], 'Q79A': [198, 211, 451, 464], 'mutation': [199, 452, 1797, 1861], 'abolished': [200, 453], 'full-length': [207, 460, 4235, 4287], 'Nevertheless,': [209, 462], 'mutant': [212, 465, 2886, 3431], 'restored': [213, 466], 'transport': [215, 468], 'NPC1-deficient': [217, 470], 'Chinese': [218, 471, 859, 1244], 'hamster': [219, 472, 617, 860, 1245, 1428], 'ovary': [220, 473, 861, 1246], 'cells.': [221, 474, 1409, 1435, 2367, 3180], 'Thus,': [222, 475], 'on': [227, 480, 3333, 4324], 'essential': [232, 485], 'function': [235, 241, 488, 494, 2054], 'it': [239, 492, 984, 1029, 1151, 1717], 'may': [240, 493, 779], 'other': [243, 496, 965, 1779, 1784, 2443], 'cells': [244, 497, 904, 1087, 1248, 1813, 1840, 1857, 2164, 2795, 2898, 2955, 2973, 3032, 3060, 3130, 3681, 3717, 3750], 'where': [245, 498, 942, 972], 'produces': [248, 501], 'more': [249, 502], 'complicated': [250, 503], 'lipid': [251, 504, 521, 662, 874], 'abnormalities.': [252, 505], 'Through': [506], 'studies': [507, 768, 2276, 2334], 'humans': [509], 'inborn': [511], 'errors': [512], 'metabolism,': [514], 'scientists': [515], 'have': [516, 1255, 1795, 1846], 'uncovered': [517], 'many': [518], 'aspects': [519], 'biochemistry': [522], '(1Brady': [523], 'R.O.': [524, 1371, 4044], 'Annu.': [525], 'Rev.': [526], 'Biochem.': [527], '1978;': [528], '47:': [529], '687-713Crossref': [530], 'PubMed': [531, 544, 559, 713, 762, 817, 898, 1022, 1055, 1077, 1145, 1164, 1182, 1231, 1278, 1382, 1464, 1533, 1578, 1612, 1650, 1678, 1711, 1744, 1769, 1834, 1885, 1923, 1946, 1969, 1995, 2034, 2104, 2138, 2200, 2228, 2326, 2482, 2633, 2781, 2857, 2935, 3103, 3403, 3578, 3832, 3898, 3951, 4055, 4099, 4127, 4180, 4264], 'Scopus': [532, 545, 560, 714, 763, 818, 899, 1023, 1056, 1146, 1165, 1232, 1383, 1465, 1534, 1579, 1613, 1651, 1679, 1712, 1745, 1770, 1835, 1886, 1924, 1947, 1970, 1996, 2035, 2105, 2139, 2201, 2229, 2327, 2483, 2634, 2782, 2858, 3104, 3579, 3833, 3899, 3952, 4056, 4100, 4128, 4181, 4265], '(34)': [533], 'Google': [534, 547, 562, 716, 765, 820, 901, 1025, 1058, 1078, 1148, 1167, 1183, 1234, 1279, 1385, 1467, 1536, 1581, 1615, 1653, 1681, 1714, 1747, 1772, 1837, 1888, 1926, 1949, 1972, 1998, 2037, 2107, 2141, 2203, 2231, 2329, 2485, 2636, 2784, 2860, 2936, 3106, 3404, 3581, 3835, 3901, 3954, 4058, 4102, 4130, 4183, 4267], 'Scholar,': [535, 548, 1059, 1168, 1654, 1748, 1927, 1950, 1973, 2204, 4103], '2Goldstein': [536], 'J.L.': [537, 1008, 1050, 1133, 1567, 1600, 1639, 1699, 1733, 1758, 2126, 2186, 2216, 2314, 2470, 2621, 2769, 2845, 3091, 3567, 3820, 3888, 4088, 4252], 'Brown': [538, 1011, 1130, 1213, 1334, 1564, 1597, 1636, 1696, 1730, 1755, 2123, 2189, 2213, 2311, 2467, 2618, 2766, 2842, 3088, 3564, 3817, 3891, 4007, 4085, 4249], 'M.S.': [539, 1012, 1048, 1131, 1565, 1598, 1637, 1697, 1731, 1756, 2124, 2190, 2214, 2312, 2468, 2619, 2767, 2843, 2921, 3089, 3565, 3818, 3892, 4086, 4250], 'Science.': [540, 1051, 1227, 1378, 1830, 4051], '2001;': [541], '292:': [542], '1310-1312Crossref': [543], '(202)': [546], '3Kolter': [549], 'T.': [550, 1904, 1908], 'Sandhoff': [551], 'K.': [552, 630, 1218, 1352, 4025], 'Biochim.': [553, 892, 1158, 1917], 'Biophys.': [554, 893, 1159, 1918], 'Acta.': [555, 894, 1160, 1919], '2006;': [556, 1014, 1987, 2192], '1758:': [557], '2057-2079Crossref': [558], '(283)': [561], 'Scholar).': [563, 654, 717, 766, 821, 902, 1079, 1184, 1280, 1386, 1468, 1537, 1582, 1682, 1773, 1838, 1889, 2038, 2108, 2232, 2330, 2486, 2637, 2785, 2861, 3107, 3405, 3902, 4184, 4268], 'One': [564], 'disorder': [565, 664], 'remains': [567, 1101], 'puzzling': [568], 'complex': [571], 'known': [573, 1397], 'C,': [577], 'which': [578, 946, 999, 1400, 1477, 1499, 3233, 3779, 4220], 'caused': [580], 'loss-of-function': [582], 'mutations': [583, 2047], 'two': [587, 976, 2052], 'different': [588], 'genes,': [589], '5The': [591], 'abbreviations': [592], 'used': [593, 2900, 3034, 3800], 'are:NPC1Niemann-Pick,': [594], 'C1NPC2Niemann-Pick,': [596], 'C2ACATacyl-CoA': [598], 'acyltransferaseCMCcritical': [600], 'micellar': [601, 832, 2301], 'concentrationEndoHendoglycosidase': [602], 'HCMVcytomegalovirusDDMn-dodecyl-β-d-maltopyranosideDHPC1,2-diheptanoyl-sn-glycero-3-phosphocholineERendoplasmic': [603], 'reticulum25-HC25-hydroxycholesterolLPDSlipoprotein-deficient': [604], 'serumSREBPsterol': [605], 'regulatory': [606, 845, 977, 995, 2172], 'element-binding': [607, 846, 996], 'proteinβ-VLDLβ-migrating': [608], 'very': [609, 849], 'low': [610, 850, 853, 926], 'density': [611, 613, 851, 854, 927], 'lipoproteinLDLlow': [612], 'lipoproteinBSAbovine': [614], 'serum': [615, 843, 857, 2424, 2439, 2827, 2984], 'albuminCHOChinese': [616], 'ovaryCHAPS3-[(3-cholamidopropyl)dimethylammonio]-1-propane-sulfonic': [618], 'acidHSVherpes': [619], 'simplex': [620, 865, 2750], 'virusNi-NTAnickel-nitrilotriacetic': [621], 'acid.': [622, 868], '(4Pentchev': [625], 'P.G.': [626, 891, 1157, 1224, 1375, 1518, 4048, 4165], 'Vanier': [627, 1372, 1822, 2088, 4045], 'M.T.': [628, 705, 754, 1373, 1823, 2089, 4046], 'Suzuki': [629], 'Patterson': [631, 1368, 4041], 'M.C.': [632, 1369, 4042], 'Scriver': [633], 'C.R.': [634, 1337, 4010], 'Beaudet': [635], 'A.L.': [636, 3391], 'Sly': [637], 'W.S.': [638], 'Valle': [639], 'D.': [640, 1309, 1360, 3982, 4033], 'The': [641, 655, 718, 955, 1469, 2039, 2675, 2718, 2733, 2950, 3296, 3324, 3344, 3406, 3535, 3926, 4226, 4292], 'Metabolic': [642], 'Molecular': [644], 'Basis': [645], 'Inherited': [647], 'Disease.': [648], 'McGraw-Hill': [649], 'Inc.,': [650], 'New': [651], 'York1995:': [652], '2625-2639Google': [653], 'clinical': [656], 'spectrum': [657], 'patients': [659, 1791], 'this': [661, 784, 1112, 1685, 1775, 2380, 4320], 'storage': [663, 1041], 'wide.': [666], 'Most': [667], 'subjects': [668, 1842], 'manifest': [669], 'childhood': [671], 'devastating': [674], 'syndrome': [675], 'includes': [677, 1495], 'enlargement': [678], 'liver': [680, 719], 'lung': [683], 'disease,': [684], 'neurological': [686], 'degeneration': [687], 'manifested': [688], 'ataxia,': [690], 'dysarthria,': [691], 'gaze': [692], 'palsies,': [693], 'progressive': [695], 'dementia,': [696], 'usually': [697], 'resulting': [698], 'death': [700], 'before': [701], 'age': [702], '20': [703, 3276], '(5Vanier': [704, 753], 'Millat': [706, 755], 'G.': [707, 756], 'Clin.': [708, 757], 'Genet.': [709, 758], '2003;': [710, 759, 1877, 1943, 1966], '64:': [711, 760], '269-281Crossref': [712, 761], '(492)': [715, 764], 'spleen': [721], 'unesterified': [723, 956], 'sphingomyelin,': [725, 744], 'variety': [728, 872], 'phospholipids': [730], 'glycosphingolipids.': [732], 'Brain': [733], 'tissue': [734], 'does': [735], 'contain': [737, 3931, 4298], 'gross': [739, 790], 'excess': [740, 781], 'or': [743, 1783, 2251, 2888, 3069, 3301, 3318, 3360, 3382, 3454, 3477, 3484, 3660, 3667, 3740, 4203], 'there': [746, 2240], 'distinct': [749], 'elevation': [750], 'glycosphingolipids': [752], 'Recent': [767], 'mouse': [771], 'model': [772], 'indicate': [776], 'neurons': [778], 'accumulation': [785], 'masked': [787], 'at': [788, 2493, 2510, 2526, 2539, 2985, 3190, 3196, 3213, 3255, 3487, 3647, 3744, 4145], 'level': [791], 'decline': [794], 'amount': [797, 3620, 3653], 'myelin': [801], '(6Xie': [802], 'C.': [803, 1212, 1311, 3244, 3343, 3984], 'Burns': [804], 'D.K.': [805], 'Turley': [806], 'S.D.': [807], 'Dietschy': [808], 'J.M.': [809], 'J.': [810, 1068, 1134, 1173, 1216, 1269, 1313, 1321, 1339, 1358, 1453, 1522, 1596, 1601, 1662, 1667, 1695, 1700, 1874, 1931, 1983, 1984, 2023, 2127, 2217, 2315, 2471, 2622, 2765, 2770, 2846, 2926, 3092, 3394, 3821, 3940, 3986, 3994, 4012, 4031, 4111, 4116, 4169, 4253], 'Neuropathol.': [811], 'Exp.': [812], 'Neurol.': [813], '2000;': [814, 1456, 1831, 3943], '59:': [815], '1106-1117Crossref': [816], '(97)': [819, 1535, 4182], 'C1': [824], 'C2': [827], 'acyl-CoA': [828], 'acyltransferase': [830, 1033], 'critical': [831], 'concentration': [833, 2297], 'endoglycosidase': [834], 'H': [835], 'cytomegalovirus': [836, 2597], 'n-dodecyl-β-d-maltopyranoside': [837, 2415], '1,2-diheptanoyl-sn-glycero-3-phosphocholine': [838], 'endoplasmic': [839, 969], 'reticulum': [840, 970], '25-hydroxycholesterol': [841, 2159], 'lipoprotein-deficient': [842, 2438], 'β-migrating': [848], 'lipoprotein': [852, 855, 928], 'bovine': [856, 2423], 'albumin': [858, 2425], '3-[(3-cholamidopropyl)dimethylammonio]-1-propane-sulfonic': [862], 'herpes': [864, 2749], 'virus': [866, 2751], 'nickel-nitrilotriacetic': [867], 'Despite': [869], 'wide': [871], 'abnormalities': [875], 'organs': [877], 'these': [879, 2167, 4271, 4295], 'patients,': [880, 1084], 'functional': [882], 'abnormality': [883], 'fibroblasts': [886, 1081, 1190], 'surprisingly': [888], 'simple': [889], '(7Pentchev': [890, 1156], '2004;': [895, 1161, 1736, 2101, 3570], '1685:': [896, 1162], '3-7Crossref': [897, 1163], '(72)': [900, 1166], 'These': [903], 'exhibit': [905], 'striking': [907], 'defect': [908, 1187, 1257, 1421, 1849], 'disposal': [911], 'has': [915, 1471, 1718, 1891, 2010, 2070], 'entered': [916], 'cell': [918, 3880], 'through': [919, 3202, 3258], 'endocytosis': [921], 'initiated': [922], '(LDL)': [929], 'receptors.': [930], 'After': [931, 2893, 3013, 3028, 3151, 3181, 3480, 3742], 'coated': [934], 'vesicles,': [935], 'ingested': [936], 'LDL': [937, 1085], 'delivered': [939, 2162, 3458, 3605], 'esters,': [945], 'constitute': [947], 'bulk': [949], 'its': [951], 'are': [953, 1000, 1093, 1417, 1585, 3801], 'hydrolyzed.': [954], 'exits': [958], 'lysosome': [960], 'transported': [963], 'organelles,': [966], 'including': [967], '(ER),': [971], 'exerts': [975], 'actions': [978, 2069, 2173, 2248], 'maintain': [980], 'homeostasis:': [982], '1)': [983, 3965], 'blocks': [985], 'ER': [987, 1561, 1589, 2171], 'exit': [988, 1113], 'subsequent': [990], 'proteolytic': [991], 'activation': [992], 'proteins': [997, 1780, 2053, 2905, 3055, 3125, 3408, 4228, 4274, 4297], '(SREBPs),': [998], 'transcription': [1001], 'factors': [1002], 'increase': [1004], 'synthesis': [1006], '(8Goldstein': [1007, 2185], 'DeBose-Boyd': [1009, 2187], 'R.A.': [1010, 2188], 'Cell.': [1013, 1735, 2191, 3569], '124:': [1015, 2193], '35-46Abstract': [1016, 2194], 'Full': [1017, 1019, 1074, 1140, 1142, 1179, 1275, 1459, 1461, 1528, 1530, 1607, 1609, 1673, 1675, 1706, 1708, 1739, 1741, 1880, 1882, 1990, 1992, 2029, 2031, 2133, 2135, 2195, 2197, 2223, 2225, 2321, 2323, 2477, 2479, 2628, 2630, 2776, 2778, 2852, 2854, 2932, 3098, 3100, 3400, 3573, 3575, 3827, 3829, 3946, 3948, 4122, 4124, 4175, 4177, 4259, 4261], 'Text': [1018, 1020, 1075, 1141, 1143, 1180, 1276, 1460, 1462, 1529, 1531, 1608, 1610, 1674, 1676, 1707, 1709, 1740, 1742, 1881, 1883, 1991, 1993, 2030, 2032, 2134, 2136, 2196, 2198, 2224, 2226, 2322, 2324, 2478, 2480, 2629, 2631, 2777, 2779, 2853, 2855, 2933, 3099, 3101, 3401, 3574, 3576, 3828, 3830, 3947, 3949, 4123, 4125, 4176, 4178, 4260, 4262], 'PDF': [1021, 1076, 1144, 1181, 1277, 1463, 1532, 1611, 1677, 1710, 1743, 1884, 1994, 2033, 2137, 2199, 2227, 2325, 2481, 2632, 2780, 2856, 2934, 3102, 3402, 3577, 3831, 3950, 4126, 4179, 4263], '(1236)': [1024, 2202], 'Scholar),': [1026, 1149, 1235, 1715, 1999, 2142, 2937], '2)': [1028, 4060], 'activates': [1030], 'acyl-CoA:': [1031], '(ACAT),': [1034], 'an': [1035, 1200, 1859, 2958, 3203, 3314], 'enzyme': [1036, 1590], 're-esterifies': [1038], 'cytoplasmic': [1043, 1491], 'ester': [1045], 'droplets': [1046], '(9Brown': [1047], 'Goldstein': [1049, 1132, 1566, 1599, 1638, 1698, 1732, 1757, 2125, 2215, 2313, 2469, 2620, 2768, 2844, 3090, 3566, 3819, 4087, 4251], '1986;': [1052], '232:': [1053], '34-47Crossref': [1054], '(4362)': [1057], '10Chang': [1060], 'C.C.Y.': [1061], 'Huh': [1062], 'H.Y.': [1063], 'Cadigan': [1064], 'K.M.': [1065, 1264], 'Chang': [1066, 1267, 1344, 4017], 'T.Y.': [1067], 'Biol.': [1069, 1135, 1174, 1270, 1454, 1523, 1602, 1668, 1701, 1875, 1985, 2024, 2128, 2218, 2316, 2472, 2623, 2771, 2847, 2927, 3093, 3395, 3822, 3941, 4117, 4170, 4254], 'Chem.': [1070, 1136, 1175, 1271, 1455, 1524, 1603, 1669, 1702, 1876, 1986, 2025, 2129, 2219, 2317, 2473, 2624, 2772, 2848, 2928, 3094, 3396, 3823, 3942, 4118, 4171, 4255], '1993;': [1071], '268:': [1072], '20747-20755Abstract': [1073], 'from': [1082, 1191, 1261, 1841, 2062, 2405, 2413, 2419, 2427, 2450, 2641, 2662, 2722, 2940, 2957, 3050, 3116], 'NPC': [1083, 1201, 1287, 1793], 'enters': [1086], 'normally,': [1088], 'esters': [1092], 'hydrolyzed': [1094], 'lysosomes,': [1096, 1808, 2063, 2238], 'liberated': [1099], 'trapped': [1102], 'free': [1104], 'As': [1108, 2109], 'result': [1110], 'block,': [1114], 'LDL-derived': [1115], 'fails': [1117, 1152], 'inhibit': [1119], 'SREBP': [1120, 2179], 'processing': [1121], '(11Infante': [1122, 2115, 2303, 2459, 2610, 2834, 3080, 3809, 4241], 'R.E.': [1123, 2116, 2206, 2304, 2460, 2611, 2835, 3081, 3810, 4242], 'Abi-Mosleh': [1124, 2117, 2207, 2305, 2461, 2612, 2836, 3082, 3811, 4243], 'L.': [1125, 1170, 1347, 2083, 2118, 2208, 2306, 2462, 2613, 2837, 2925, 3083, 3812, 4020, 4244], 'Radhakrishnan': [1126, 2119, 2209, 2307, 2463, 2614, 2838, 3084, 3813, 4245], 'A.': [1127, 1214, 1333, 1335, 1563, 1574, 1635, 1646, 1725, 1750, 1765, 1821, 1863, 1933, 1942, 1965, 2100, 2120, 2210, 2308, 2464, 2615, 2839, 3085, 3559, 3814, 4006, 4008, 4084, 4095, 4246], 'Dale': [1128, 2121, 2211, 2309, 2465, 2616, 2840, 3086, 3815, 4247], 'J.D.': [1129, 2122, 2212, 2310, 2466, 2617, 2841, 3087, 3816, 4248], '2007;': [1137, 1766, 2026, 2130, 2220, 2318, 2474, 2625, 2849, 3095, 3824, 4256], '283:': [1138, 2131, 2221, 2319, 2475, 2626, 2850, 3096, 3825, 4257], '1052-1063Abstract': [1139, 2132, 2222, 2320, 2476, 2627, 2851, 3097, 3826, 4258], '(172)': [1147, 2140, 2230, 2328, 2484, 2635, 2859, 3105, 3834, 4266], 'activate': [1154, 2183], 'ACAT': [1155, 2184, 3038, 3866], '12Liscum': [1169], 'Faust': [1171, 2922], 'J.R.': [1172, 1867, 2923], '1987;': [1176], '262:': [1177], '17002-17008Abstract': [1178], 'A': [1185, 1281, 2488, 2802, 2979, 3022, 3068, 3143, 3161, 3699, 3721], 'occurs': [1188], 'BALB/c': [1192, 1423], 'npcnih': [1193, 1424], 'mice,': [1194, 1425], 'line': [1196, 1241], 'mice': [1198], 'phenotype': [1202], '(13Loftus': [1203], 'S.K.': [1204, 1307, 3890, 3980], 'Morris': [1205, 1302, 3975], 'J.A.': [1206, 1303, 2077, 3976], 'Carstea': [1207], 'E.D.': [1208, 1301, 3974], 'Gu': [1209, 1312, 3985], 'J.Z.': [1210], 'Cummings': [1211, 1310, 3983], 'Ellison': [1215], 'Ohno': [1217, 1350, 4023], 'Rosenfeld': [1219, 1314, 3987], 'M.A.': [1220, 1315, 3988], 'Tagle': [1221, 1376, 4049], 'D.A.': [1222, 1377, 4050], 'Pentchev': [1223, 1374, 1517, 4047, 4164], 'Pavan': [1225, 1316, 3989], 'W.J.': [1226, 1317, 3990], '1997;': [1228, 1379, 4052], '277:': [1229, 1380, 4053], '232-235Crossref': [1230], '(699)': [1233], 'CT60': [1238], '(CHO)': [1247], 'were': [1250, 2258, 2448, 2692, 2796, 2899, 2938, 2974, 3033, 3056, 3126, 3222, 3368, 3409, 3592, 3685, 3751, 3766, 3780, 4229, 4281], 'mutagenized': [1251], 'selected': [1253], 'release': [1260, 1851], '(14Cadigan': [1263], 'Heider': [1265], 'J.G.': [1266], 'T.-Y.': [1268, 1345, 4018], '1988;': [1272], '263:': [1273], '274-282Abstract': [1274], 'major': [1282], 'advance': [1283], 'understanding': [1285], 'occurred': [1289, 2292], 'when': [1290, 2294], 'first': [1292, 4199], 'defective': [1293, 1431, 2913, 2951], 'gene': [1294, 1388], 'identified': [1296], 'positional': [1298], 'cloning': [1299], '(15Carstea': [1300, 3973], 'Coleman': [1304, 3977], 'K.G.': [1305, 3978], 'Loftus': [1306, 3979], 'Zhang': [1308, 1661, 3981, 4110], 'Krizman': [1318, 3991], 'D.B.': [1319, 3992], 'Nagle': [1320, 3993], 'Polymeropoulos': [1322, 3995], 'M.H.': [1323, 3996], 'Sturley': [1324, 3997], 'S.L.': [1325, 3998], 'Ioannou': [1326, 1451, 3938, 3999], 'Y.A.': [1327, 1452, 3939, 4000], 'Higgins': [1328, 4001], 'M.E.': [1329, 4002], 'Comly': [1330, 4003], 'M.': [1331, 1354, 1367, 1512, 1827, 1902, 2079, 4004, 4027, 4040, 4159], 'Cooney': [1332, 4005], 'Kaneski': [1336, 4009], 'Blanchette-Mackie': [1338, 1507, 4011, 4154], 'Dwyer': [1340, 1509, 4013, 4156], 'N.K.': [1341, 1510, 2917, 4014, 4157], 'Neufeld': [1342, 1513, 4015, 4160], 'E.B.': [1343, 1514, 4016, 4161], 'Liscum': [1346, 2924, 2942, 4019], 'Strauss': [1348, 1519, 4021, 4166], 'J.F.': [1349, 1521, 4022, 4168], 'III,': [1351, 1520, 4024, 4167], 'Zeigler': [1353, 4026], 'Carmi': [1355, 4028], 'R.': [1356, 1825, 1979, 4029], 'Sokol': [1357, 4030], 'Markie': [1359, 4032], "O'Neill": [1361, 4034], 'R.R.': [1362, 4035], 'van': [1363, 4036], 'Diggelen': [1364, 4037], 'O.P.': [1365, 4038], 'Elleder': [1366, 4039], 'Brady': [1370, 4043], '228-231Crossref': [1381, 4054], '(1215)': [1384, 4057], 'encodes': [1389, 2580, 2703, 2747], '1278-amino': [1391, 3923], 'integral': [1393], 'membrane': [1394, 1443, 3777], 'protein,': [1395], 'now': [1396], 'NPC1,': [1399, 3921], 'resides': [1401], 'late': [1403], 'endosomes': [1404], 'Mutations': [1410, 2689], 'murine': [1413], 'version': [1414, 1429], 'responsible': [1418], 'CT': [1433], '60': [1434], 'Evidence': [1436], 'indicates': [1437], 'polytopic': [1442], '(16Davies': [1449, 3936], 'J.P.': [1450, 3937], '275:': [1457, 3944], '24367-24374Abstract': [1458, 3945], '(234)': [1466, 3953], 'cleaved': [1473, 3967], 'N-terminal': [1474], 'signal': [1475, 3968, 4143], 'sequence,': [1476, 1498], 'targets': [1478, 1500], 'insertion': [1482], 'membranes,': [1484], 'loops,': [1488], 'C-terminal': [1492, 4216], 'segment': [1493], 'dileucine': [1497], '(17Watari': [1505, 4152], 'H.': [1506, 1819, 4153], 'E.J.': [1508, 4155], 'Watari': [1511, 4158], 'Patel': [1515, 4162], 'S.': [1516, 1573, 1645, 1764, 1815, 1900, 1906, 1941, 1964, 2014, 2099, 4094, 4163], '1999;': [1525, 1920, 4172], '274:': [1526, 4173], '21861-21866Abstract': [1527, 4174], 'Helices': [1538], '3-7': [1539], 'mature': [1541], 'bear': [1543], 'sequence': [1544, 1549, 1686, 1776, 3969], 'homology': [1545], 'sterol-sensing': [1548, 1557, 1690, 4075], 'found': [1552, 1586], 'initially': [1553], 'Scap,': [1555, 1684], '(18Nohturfft': [1562, 1634, 4083], 'Proc.': [1568, 1640, 1759, 1936, 1959, 2094, 4089], 'Natl.': [1569, 1641, 1760, 1937, 1960, 2095, 4090], 'Acad.': [1570, 1642, 1761, 1938, 1961, 2096, 4091], 'Sci.': [1571, 1643, 1762, 1939, 1962, 2097, 4092], 'U.': [1572, 1644, 1763, 1940, 1963, 2098, 4093], '1998;': [1575, 1647, 4096], '95:': [1576, 1648, 4097], '12848-12853Crossref': [1577, 1649, 4098], '(111)': [1580, 1652, 4101], 'Similar': [1583], 'sequences': [1584], '3-hydroxy-3-methylglutaryl-CoA': [1591], 'reductase': [1592], '(19Hua': [1593, 1692, 2762], 'X.': [1594, 1693, 2763], 'Sakai': [1595, 1694, 2764], '1996;': [1604, 1703, 2773], '271:': [1605, 1704, 2774], '10379-10384Abstract': [1606, 1705, 2775], '(170)': [1614, 1713, 2783], 'Scholar)': [1616, 3836, 3955], 'Patched,': [1619], 'receptor': [1621], 'Hedgehog,': [1623], 'morphogen': [1626], 'bears': [1628], 'covalently': [1630], 'attached': [1631], 'moiety': [1633], '20Millard': [1655, 4104], 'E.E.': [1656, 4105], 'Gale': [1657, 4106], 'S.E.': [1658, 1865, 4107], 'Dudley': [1659, 4108], 'N.': [1660, 1869, 1898, 1952, 4109], 'Schaffer': [1663, 1870, 4112], 'J.E.': [1664, 1871, 4113], 'Ory': [1665, 1872, 4114], 'D.S.': [1666, 1873, 4115], '2005;': [1670, 4119], '280:': [1671, 4120], '28581-28590Abstract': [1672, 4121], '(84)': [1680, 4129], 'named': [1688], 'been': [1719, 1892, 2011, 2072, 3507], 'shown': [1720], 'bind': [1722, 1895], '(21Radhakrishnan': [1724, 3558], 'Sun': [1726, 3560], 'L.-P.': [1727, 3561], 'Kwon': [1728, 1753, 3562], 'H.J.': [1729, 1754, 3563], 'Mol.': [1734, 3568], '15:': [1737, 3571], '259-268Abstract': [1738, 3572], '(260)': [1746, 3580], '22Radhakrishnan': [1749], 'Ikeda': [1751], 'Y.': [1752, 1914], '104:': [1767], '6511-6518Crossref': [1768], '(435)': [1771], 'Whether': [1774], 'unknown.': [1787], 'Approximately': [1788], '5-10%': [1789], 'partially': [1805, 1810], 'sequestered': [1806], '(23Naureckiene': [1814], 'Sleat': [1816], 'D.E.': [1817, 2075], 'Lackland': [1818], 'Fensom': [1820], 'Wattiaux': [1824], 'Jadot': [1826], 'Lobel': [1828, 1955, 1980, 2019, 2092], 'P.': [1829, 1956, 1981, 2020, 2093], '290:': [1832], '2298-2301Crossref': [1833], '(701)': [1836], 'Cultured': [1839, 3677], 'same': [1848, 3619], 'do': [1856, 2235], '(24Frolov': [1862], 'Zielinski': [1864], 'Crowley': [1866], 'Dudley-Rucker': [1868], '278:': [1878], '25517-25525Abstract': [1879], '(166)': [1887], 'demonstrated': [1893], '(25Okamura': [1897], 'Kiuchi': [1899], 'Tamba': [1901], 'Kashima': [1903], 'Hiramoto': [1905], 'Baba': [1907], 'Dacheux': [1909, 1911], 'F.': [1910, 3673], 'J.-L.': [1912], 'Sugita': [1913], 'Jin': [1915], 'Y.-Z.': [1916], '1438:': [1921], '377-387Crossref': [1922], '(165)': [1925], '26Ko': [1928], 'D.C.': [1929], 'Binkley': [1930], 'Sidow': [1932], 'Scott': [1934], 'M.P.': [1935], '100:': [1944, 1967], '2518-2525Crossref': [1945], '(161)': [1948], '27Friedland': [1951], 'Liou': [1953, 2017], 'H.-L.': [1954, 2018], 'Stock': [1957, 2021], 'A.M.': [1958, 2022], '2512-2517Crossref': [1968], '(255)': [1971], '28Cheruku': [1974], 'S.R.': [1975], 'Xu': [1976], 'Z.': [1977], 'Dutia': [1978], 'Storch': [1982], '281:': [1988], '31594-31604Abstract': [1989], '(153)': [1997], 'crystal': [2002], 'structure': [2003, 3918], 'solved': [2012], '(29Xu': [2013], 'Benoff': [2015], 'B.': [2016], '282:': [2027], '23525-23531Abstract': [2028], '(164)': [2036], 'similarity': [2040], 'phenotypes': [2042], 'between': [2043, 3353, 3361], 'suggests': [2049], 'sequentially': [2055, 2585, 2708, 3268], 'pathway': [2058], 'releases': [2060], 'order': [2066], 'their': [2068, 3371], 'determined': [2073, 4327], '(30Sleat': [2074], 'Wiseman': [2076], 'El-Banna': [2078], 'Price': [2080], 'S.M.': [2081], 'Verot': [2082], 'Shen': [2084], 'M.M.': [2085], 'Tint': [2086], 'G.S.': [2087], 'Walkley': [2090], 'S.U.': [2091], '101:': [2102], '5886-5891Crossref': [2103], '(271)': [2106], 'described': [2110, 2454, 2605, 2829, 2907, 3062, 3079, 3132, 3531, 3554, 3688, 3804, 3886, 4232], 'accompanying': [2113, 2457, 2608, 2832, 3807, 4239], 'report': [2114, 2458, 2609, 2833, 3808, 4240], 'encountered': [2144], 'unexpectedly': [2146], 'search': [2149], 'membrane-bound': [2152], 'such': [2157], '(25-HC).': [2160], 'ethanol,': [2166], 'mimic': [2169], 'i.e.': [2176], 'they': [2177, 2182, 2245], 'block': [2178], 'cleavage': [2180], '11Infante': [2205], 'However,': [2233], 'traverse': [2237], 'no': [2242], 'evidence': [2243, 2385], 'require': [2246], 'reach': [2254], 'ER.': [2256], 'therefore': [2259], 'surprised': [2260], 'find': [2262], 'purified': [2265, 3065, 3655, 4230], 'membrane-bound,': [2266], 'oxysterol-binding': [2267], 'turned': [2269], 'out': [2270, 3594, 3635], 'be': [2272], 'further': [2275], 'recombinant': [2280, 2348], 'human': [2281, 2342, 2582, 2705, 2753, 3920], 'well': [2286], 'oxysterols,': [2288], 'only': [2293], 'detergent': [2296], 'reduced': [2299], 'below': [2300], 'levels': [2302], 'current': [2333], 'loop-1.': [2346], 'Using': [2347], 'DNA': [2349], 'technology,': [2350], 'prepare': [2352], '240': [2360], 'Purified': [2368], 'high': [2376], 'affinity.': [2377], 'characterize': [2379], 'reaction': [2382, 3438], 'provide': [2384], 'majority,': [2391], 'if': [2392], 'all,': [2394], 'cholesterol-binding': [2397], 'activity': [2398, 3350], 'Materials—We': [2401], 'obtained': [2402, 2449, 2939], '1,2-diheptanoyl-SN-glycero-3-phosphocholine': [2403], '(DHPC)': [2404], 'Avanti': [2406], 'Polar': [2407], 'Lipids,': [2408], 'Inc.;': [2409, 2421], 'sodium': [2410, 2553, 3711, 3733], 'Sigma;': [2414], '(DDM)': [2416], 'CHAPS': [2418], 'Anatrace,': [2420], '(BSA)': [2426], 'Pierce': [2428], '(Cat.': [2429], 'No.': [2430], '23210).': [2431], 'Radioisotopes,': [2432], 'antibodies,': [2433], 'sterols,': [2434, 3588], 'chromatographic': [2435], 'supplies,': [2436], 'lipoproteins,': [2437], '(LPDS),': [2440], 'all': [2442, 3615], 'reagents': [2444], '(unless': [2445], 'otherwise': [2446], 'specified)': [2447], 'sources': [2451], 'prepared': [2452], 'Buffers—Buffer': [2487], '50': [2490, 2507, 2523, 2536, 2542, 3270, 3287, 3709, 3728, 3731], 'mm': [2491, 2497, 2508, 2514, 2524, 2531, 2537, 2543, 2549, 2552, 3277, 3280, 3294, 3542], 'Tris-chloride': [2492, 2509, 2525, 2538], 'pH': [2494, 2511, 2527, 2540], '7.4,': [2495, 2512, 2541], '150': [2496, 2530], 'NaCl,': [2498], '0.004%': [2500], '(w/v)': [2501], 'Nonidet': [2502, 2518], 'P-40.': [2503, 2519], 'Buffer': [2504, 2520, 2533], 'B': [2505, 3070, 3546], '100': [2513, 2814, 2818], 'KCl,': [2515, 2544], '1%': [2517, 3145, 3163], 'C': [2521, 3274, 3291, 4147], '7.4': [2528], 'NaCl.': [2532], 'D': [2534, 3860], '10%': [2545], '(v/v)': [2546, 2824, 3146], 'glycerol,': [2547], '5': [2548, 2872, 2876, 2880, 2889, 3014, 3194, 3705, 3748], 'dithiothreitol,': [2550], '1': [2551, 3466, 3759], 'EDTA,': [2554], 'protease': [2556], 'inhibitor': [2557], 'mixture': [2558, 2804, 3491], '(1': [2559, 3247], 'μg/ml': [2560, 2564, 2567, 2575, 2819, 3755, 3851], 'pepstatin': [2561], 'A,': [2562, 3449], '2': [2563, 2986], 'aprotinin,': [2565], 'leupeptin,': [2568], '200': [2569, 3293], 'μm': [2570, 3706, 3710, 3729, 3732], 'phenylmethylsulfonyl': [2571], 'fluoride,': [2572], '25': [2574, 3754], 'N-acetyl-Leu-Leu-norleucinal).': [2577], 'Plasmid': [2578], 'Constructions—pCMV-NPC1-His8-FLAG': [2579], 'wild-type': [2581, 2704, 2748, 3429], 'followed': [2584, 2707, 3413], 'eight': [2587, 2710], 'histidines': [2588, 2711], 'FLAG': [2591], 'tag': [2592], 'under': [2593, 2712, 2755], 'control': [2594, 2713, 2756], '(CMV)': [2598], 'promoter.': [2599, 2717], 'plasmid': [2601, 2680, 2719], 'constructed': [2603, 2640, 2661, 2721], 'pCMV-NPC1-(1-307;1254-1278)-His8-FLAG': [2638, 2663], 'pCMV-NPC1-His8-FLAG': [2642, 2700], 'site-directed': [2644, 2665, 2695, 2727], 'mutagenesis': [2645, 2666, 2696, 2728], '(QuikChange': [2646, 2729], 'II': [2647, 2730], 'XL': [2648, 2731], 'kit,': [2649], 'Stratagene)': [2650], 'using': [2651, 2667, 3005, 3309], '5′-oligonucleotide,': [2653, 2669], '5′-CTCCGAGTACACTCCCATCGATAGCGTAAATAAAGCCAAAAGTTGTGCC-3′': [2654], '3′-oligonucleotide,': [2657, 2673], '5′-GGCACAACTTTTGGCTTTATTTACGCTATCGATGGGAGTGTAGTCGGA-3′.': [2658], 'pCMV-NPC1-(1-264)-His8-FLAG': [2659, 2698], '5′-CCTGCTCCCTGGACGATCCTTGGCCATCACCATCACCATCACCATCACGACTAA-3′': [2670], '5′-TTATAGTCGTGATGGTGATGGTGATGGTGATGGCCAAGGATCGTCCAGGGAGCAGG-3′.': [2674], 'coding': [2676, 2734], 'region': [2677, 2735, 4188], 'each': [2679, 2994, 3176, 3182, 3223, 3234, 3259, 3437, 3613], 'sequenced': [2682, 2739], 'ensure': [2684, 2741], 'integrity': [2685, 2742], 'construct.': [2688, 2745], 'plasmids.': [2701], 'pCMV-NPC2-His8': [2702, 2737], 'CMV': [2716], 'pCMV-NPC2': [2723], '(Origene': [2724], 'Technologies)': [2725], 'kit).': [2732], 'pTK-HSV-BP-2': [2746], '(HSV)-tagged': [2752], 'SREBP-2': [2754, 3040, 3674, 3786, 3846], 'thymidine': [2759], 'kinase': [2760], 'promoter': [2761], 'Transfection': [2786], 'Constructs': [2791], 'CHO': [2793, 2910, 3052, 3120, 3679], 'Cells—CHO-K1': [2794], 'grown': [2797], 'transfected': [2799, 2865, 2897, 2997, 3179, 3686], 'medium': [2801, 2808, 2978, 3017, 3021, 3138, 3142, 3155, 3160, 3185, 3246, 3694, 3698, 3720], '(1:1': [2803], "Ham's": [2806], 'F-12': [2807], "Dulbecco's": [2810], 'modified': [2811], "Eagle's": [2812], 'medium,': [2813], 'units/ml': [2815], 'penicillin,': [2816], 'strep-tomycin': [2820], 'sulfate)': [2821], 'containing': [2822, 2980, 3023, 3144, 3162, 3275, 3292, 3299, 3346, 3700], '5%': [2823, 2981, 3024, 3701, 3724], 'fetal': [2825, 2982], 'calf': [2826, 2983, 3026, 3703, 3726], 'Each': [2862, 3264, 3514], 'dish': [2863, 2995], 'following': [2870, 3962], 'plasmids:': [2871], 'μg': [2873, 2877, 2881, 2890, 3467], 'pCMV-NPC1-His8-FLAG,': [2875], 'pCMV-NPC1-(1-307;1254-1278)-His8-FLAG,': [2879], 'pCMV-NPC1-(1-264)-His8-FLAG(wild-typeor': [2883], 'indicated': [2885, 3003, 3600, 3652], 'versions),': [2887], 'pCMV-NPC2-His8.': [2892], '16': [2894, 3029, 3362, 3485, 3645], 'h,': [2895, 3015, 3030, 3153, 3749], 'purification': [2902], 'below.': [2908], 'Mutant': [2909], '4-4-19': [2911, 2954, 2972, 3680], '(31Dahl': [2916], 'Reed': [2918], 'K.L.': [2919], 'Daunais': [2920], '1992;': [2929], '267:': [2930], '4889-4896Abstract': [2931], 'Laura': [2941], '(Tufts': [2943], 'University': [2944], 'School': [2945], 'Medicine,': [2947], 'Boston,': [2948], 'MA).': [2949], 'results': [2956], 'substitution': [2961], '(Gly-660': [2962], 'Arg).': [2964], '6L.': [2965], 'Liscum,': [2966], 'personal': [2967], 'communication.': [2968], 'On': [2969, 2991, 3134, 3690, 3713], 'day': [2970, 2992, 3135, 3691, 3714], '0,': [2971], 'set': [2975], 'up': [2976], '×': [2987, 3775], '105': [2988], 'cells/60-mm': [2989], 'dish.': [2990], '2,': [2993], 'OPTI-MEM': [2999], '(Invitrogen)': [3000], 'plasmid,': [3004], 'Lipofectamine': [3006], '2000': [3007], 'according': [3008, 3636], "manufacturer's": [3011, 3639], 'directions.': [3012], 'switched': [3019, 3140, 3696], 'newborn': [3025, 3702, 3725], 'LPDS.': [3027], 'assays': [3036, 3591], 'processing.': [3041], 'Purification': [3042, 3108], 'Full-length': [3044], 'Internally': [3047], 'Deleted': [3048], 'NPC1-(1-307;1254-1278)': [3049], 'Transfected': [3051, 3119], 'Cells—Recombinant': [3053, 3121], 'overexpressed': [3057, 3127], 'CHO-K1': [3059, 3129], 'above': [3063], 'buffer': [3067, 3243, 3273, 3290, 3342, 3448, 3525, 3545], 'nickel': [3072], 'M2': [3074], 'anti-FLAG-agarose': [3075], 'chromatography': [3076, 3332], 'previously': [3078, 3553, 3885], 'Secreted': [3110], 'Forms': [3111], 'NPC1-(25-264)': [3113], 'Medium': [3117, 3210], 'above.': [3133, 3689], '3,': [3136, 3692], 'Cellgro®': [3147, 3164], 'ITS': [3148, 3165], '(Fisher': [3149], 'Scientific).': [3150], '24': [3152, 4217], 'collected': [3157], 'fresh': [3159, 3719], 'added.': [3167], 'done': [3170], '3': [3172], 'consecutive': [3173], 'days': [3174], 'group': [3177], 'collection,': [3183], 'subjected': [3187, 3329, 3410], 'centrifugation': [3189], '2500': [3191], 'rpm': [3192], 'min': [3195, 3520], '4': [3197, 3214, 3239, 3256, 3483, 3488], '°C': [3198, 3215, 3257, 3649, 3746], 'then': [3200, 3252, 3328, 3762, 3781], 'filtered': [3201], 'Express': [3204], 'PLUS': [3205], '0.22-μm': [3206], 'filter': [3207], 'apparatus': [3208], '(Millipore).': [3209], 'stored': [3212], 'covered': [3216], 'aluminum': [3218], 'foil.': [3219], 'Gravity': [3220], 'columns': [3221], 'filled': [3224], '20-ml': [3227], 'slurry': [3228], 'Ni-NTA-agarose': [3230, 3260, 3502], 'beads,': [3231], 'column': [3235, 3240, 3261, 3265, 3337, 3496, 3515], 'pre-equilibrated': [3237, 3340, 3508], 'volumes': [3241], 'Filtered': [3245], 'liter': [3248], 'column)': [3250], 'passed': [3253], 'overnight': [3254], '∼1': [3262], 'ml/min.': [3263], 'washed': [3267, 3517], 'ml': [3271, 3288, 3307, 3357, 3365, 3500, 3523], '40': [3279], 'imidazole.': [3281, 3295], 'Bound': [3282], 'eluted': [3285, 3297, 3539], 'fraction': [3298], 'NPC1-(25-264)-His8-FLAG': [3300], 'NPC2-His8': [3302], 'concentrated': [3304, 3325], '0.5': [3306], 'spin': [3311], 'concentrator': [3312], 'Amicon': [3315], 'Ultracel': [3316], '30K': [3317], '10K': [3319], 'Filter': [3320], 'Device': [3321], '(Millipore),': [3322], 'respectively.': [3323], 'material': [3326], 'gel-filtration': [3331], '24-ml': [3335], 'Superdex-200': [3336], 'fractions': [3345], 'peak': [3348], 'A280': [3349], 'eluting': [3352], '12.5': [3354], '15': [3356], '(for': [3358, 3366], 'NPC1-(25-264)-His8-FLAG)': [3359], 'NPC2-His8)': [3367], 'pooled,': [3369], 'content': [3373], 'quantified': [3375, 3548], 'either': [3377, 3450], 'BCA': [3379], 'kit': [3380], '(Pierce)': [3381], 'Lowry': [3384], 'method': [3385], '(32Lowry': [3386], 'O.H.': [3387], 'Rosebrough': [3388], 'N.J.': [3389], 'Farr': [3390], 'Randall': [3392], 'R.J.': [3393], '1951;': [3397], '193:': [3398], '265-275Abstract': [3399], 'pooled': [3407, 3767], 'SDS-PAGE': [3412, 4325], 'Coomassie': [3415], 'staining': [3416], 'determine': [3418], 'purity.': [3419], 'Ni-NTA-Agarose': [3420], 'Assay': [3421], '3H-Sterol': [3423], 'Binding—For': [3424], 'standard': [3426, 3590], 'assay': [3427, 3512], 'versions': [3432], 'contained,': [3439], 'final': [3442, 3628], 'volume': [3443, 3629], '80': [3445], 'μl': [3446], '(165-180': [3452], 'dpm/fmol)': [3453, 3457], '(132': [3456], 'ethanol': [3460, 3607, 3609], '(final': [3461, 3608], '[3H]sterol': [3462, 3537], 'concentration,': [3463, 3610], '10-400': [3464], 'nm),': [3465], 'BSA': [3469], 'indicated,': [3471], 'varying': [3473], 'amounts': [3474], 'protein.': [3479, 3925], 'incubation': [3481, 3743], 'h': [3486, 3646, 3760], '°C,': [3489], 'loaded': [3493], 'onto': [3494], 'packed': [3497], '0.3': [3499], 'beads': [3503], '(Qiagen)': [3504], 'had': [3506], 'appropriate': [3511], 'buffer.': [3513], '∼15': [3519], '5-6': [3522], 'B,': [3526], 'except': [3527, 3837], 'experiments': [3530, 3585], 'Fig.': [3533, 4312, 4314], '6.': [3534], 'protein-bound': [3536], '250': [3541], 'imidazole': [3543], 'scintillation': [3550], 'counting': [3551], '(Radhakrishnan': [3555], 'et': [3556], 'al.': [3557], 'Scholar)).': [3582], 'For': [3583], 'competition': [3584], 'unlabeled': [3587, 3601], 'carried': [3593, 3634], 'presence': [3597, 3659], '(0-3': [3603], 'μm)': [3604], '1-4%).': [3611], 'experiment,': [3614], 'tubes': [3616], 'received': [3617, 3718], 'ethanol.': [3622], 'Glycosidase': [3623], 'Treatments—Each': [3624], 'reaction,': [3625], '42': [3631], 'μl,': [3632], 'directions': [3640], '(New': [3641], 'England': [3642], 'Biolabs)': [3643], '37': [3648, 3745], 'absence': [3661], '4000': [3663], 'units': [3664, 3669], 'EndoH': [3666], '5000': [3668], 'peptide': [3671], 'N-glycosidase': [3672], 'Processing': [3675], 'Cells—Mutant': [3678], '(defective': [3682], 'NPC1)': [3684], 'LPDS,': [3704, 3727], 'compactin,': [3707, 3730], 'mevalonate.': [3712], '4,': [3715], 'supplemented': [3722], 'mevalonate,': [3734], 'various': [3736], 'concentrations': [3737, 3797], 'β-VLDL': [3739], '25-HC.': [3741], 'treated': [3752], 'N-acetyl-Leu-Leu-norleucinal': [3757], 'harvested.': [3763], 'Duplicate': [3764], 'dishes': [3765], 'preparation': [3769], 'nuclear': [3771], 'extract': [3772], '100,000': [3774], 'g': [3776], 'fractions,': [3778], 'analyzed': [3782], 'immunoblotting': [3784], '(described': [3787], 'below).': [3788], 'Immunoblot': [3789], 'Analysis—The': [3790], 'procedure': [3791], 'immunoblot': [3793], 'analysis': [3794], 'antibodies': [3799], 'exactly': [3802], 'use': [3840], 'additional': [3843], 'antibody.': [3844], 'Epitope-tagged': [3845], 'detected': [3848], '0.2': [3850], 'IgG-HSV-Tag™,': [3852], 'monoclonal': [3854], 'antibody': [3855], 'directed': [3856], 'against': [3857], 'glycoprotein': [3859], 'epitope': [3861], 'HSV': [3863], '(Novagen,': [3864], 'Inc).': [3865], 'Assay—The': [3867], 'rate': [3868], 'incorporation': [3870], '[14C]oleate': [3872, 3875], '[14C]triglycerides': [3877], 'intact': [3879], 'monolayers': [3881], 'measured': [3883], '(33Goldstein': [3887], 'Basu': [3889], 'Methods': [3893], 'Enzymol.': [3894], '1983;': [3895], '98:': [3896], '241-260Crossref': [3897], '(1284)': [3900], 'Localization': [3903], 'Oxysterol': [3905], 'Site': [3907], 'NPC1—Fig.': [3909], '1A': [3910], 'shows': [3911, 4316], 'diagram': [3913], 'postulated': [3916], 'believed': [3929], 'putative': [3933, 4074], 'transmembrane': [3934], 'divide': [3957], 'structural': [3963], 'domains:': [3964], '(amino': [3970, 4063, 4069, 4135, 4149], '1-24)': [3972], 'Scholar);': [4059, 4131], '25-264);': [4065], '3)': [4066], 'loop-2': [4068], '371-615);': [4071], '4)': [4072], '(transmembrane': [4077], '3-7,': [4079], '616-791)': [4082], '5)': [4132], 'loop-3': [4134], '855-1098);': [4137], '6)': [4139], 'targeting': [4142, 4224], '(LLNF)': [4144], 'terminus': [4148], '1275-1278)': [4151], 'To': [4185], '25-HC,': [4193], 'created': [4195], 'plasmids': [4196], 'encoding': [4197], '307,': [4200], '644,': [4201], '809,': [4202], '1178': [4204], 'fused': [4210], 'fragment': [4213], 'comprising': [4214], 'acids,': [4219], 'signal.': [4225], 'epitope-tagged': [4227], 'All': [4269], 'internally': [4272, 4321], 'deleted': [4273, 4296, 4322], 'saturation': [4278], 'kinetics': [4279], '(data': [4289], 'shown).': [4291], 'smallest': [4293], '332': [4299], 'NPC1-derived': [4300], 'designated': [4305], 'NPC1-His8-FLAG-(1-307;': [4306], '1254-1278)': [4307], '(see': [4308], 'bottom': [4309], 'panel': [4310], '1A).': [4313], '1B': [4315], 'migration': [4318], 'immun': [4329]}, 'cited_by_api_url': 'https://api.openalex.org/works?filter=cites:W2114350791', 'counts_by_year': [{'year': 2024, 'cited_by_count': 7}, {'year': 2023, 'cited_by_count': 7}, {'year': 2022, 'cited_by_count': 6}, {'year': 2021, 'cited_by_count': 6}, {'year': 2020, 'cited_by_count': 15}, {'year': 2019, 'cited_by_count': 11}, {'year': 2018, 'cited_by_count': 8}, {'year': 2017, 'cited_by_count': 9}, {'year': 2016, 'cited_by_count': 15}, {'year': 2015, 'cited_by_count': 7}, {'year': 2014, 'cited_by_count': 8}, {'year': 2013, 'cited_by_count': 15}, {'year': 2012, 'cited_by_count': 17}], 'updated_date': '2024-12-10T15:34:09.979784', 'created_date': '2016-06-24'}