Publication Information

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[11, 159], 'and': [12, 30, 59, 77, 104, 113, 137, 160, 178, 207, 225, 252, 261, 285, 345, 366, 456, 460, 480, 519, 575, 927, 935, 1007, 1119, 1135, 1222, 1285, 1407, 1412, 1504, 1520, 1647, 1872, 1896, 1923, 1936, 1980, 2000, 2005, 2194, 2213, 2284, 2341, 2416, 2430, 2432, 2442, 2508, 2548, 2585, 2613, 2644, 2666, 2672, 2688, 2702, 2745, 2807, 2825, 2865, 2873, 2893, 2955, 2963, 2978, 2991, 3009, 3020, 3039, 3089, 3127, 3141, 3191, 3261, 3338, 3373, 3385, 3432, 3470, 3534, 3568, 3703, 3796, 3827, 3848, 3882, 3935, 3957, 4030, 4113, 4164, 4181, 4196, 4207, 4233, 4240, 4294, 4387], 'it': [13, 161, 3686], 'contains': [14, 98, 162, 246], 'region': [16, 164, 959, 1677], 'with': [17, 165, 1785, 2160, 2468, 2510, 2572, 2583, 2588, 2620, 2638, 2647, 2727, 2769, 2791, 2809, 2819, 2863, 2868, 2902, 2956, 2976, 3076, 3173, 3208, 3219, 3230, 3555, 3566, 3608, 3653, 3699, 3765, 3930, 4211, 4315], 'extensive': [18, 166], 'homology': [19, 167, 1784], 'the': [21, 41, 86, 89, 122, 130, 169, 189, 234, 237, 270, 278, 383, 452, 462, 493, 506, 520, 569, 572, 933, 951, 955, 1004, 1198, 1231, 1259, 1263, 1413, 1506, 1509, 1515, 1526, 1634, 1669, 1673, 1726, 1732, 1779, 1793, 1859, 1862, 1878, 1884, 1887, 1905, 1990, 2001, 2053, 2149, 2161, 2178, 2197, 2200, 2204, 2228, 2236, 2249, 2256, 2277, 2315, 2321, 2367, 2391, 2413, 2459, 2482, 2485, 2492, 2511, 2544, 2549, 2614, 2662, 2695, 2786, 2886, 2914, 2957, 3028, 3071, 3174, 3320, 3335, 3341, 3380, 3435, 3485, 3489, 3498, 3502, 3508, 3511, 3519, 3549, 3552, 3569, 3577, 3583, 3609, 3612, 3667, 3680, 3692, 3700, 3717, 3758, 3766, 3769, 3777, 3780, 3802, 3829, 3845, 3852, 3873, 3876, 3891, 3911, 3931, 3945, 3974, 3984, 3987, 4012, 4044, 4052, 4072, 4099, 4114, 4123, 4129, 4133, 4146, 4175, 4202, 4219, 4226, 4250, 4279, 4289, 4305, 4319, 4323, 4350, 4360, 4375, 4398, 4410, 4417, 4451, 4461, 4474, 4477, 4482, 4486], 'sterol-sensing': [22, 170, 311, 1635, 3381], 'domains': [23, 81, 171, 229, 3337, 3382, 4111], '(SSD)': [24, 172, 1637], 'of': [25, 44, 48, 55, 61, 75, 79, 88, 125, 135, 173, 192, 196, 203, 209, 223, 227, 236, 273, 283, 369, 385, 483, 508, 512, 954, 997, 1065, 1138, 1184, 1262, 1441, 1501, 1508, 1518, 1621, 1643, 1675, 1731, 1776, 1787, 1880, 1886, 1894, 1908, 2148, 2199, 2255, 2263, 2318, 2323, 2484, 2675, 2752, 2960, 2988, 2997, 3014, 3018, 3023, 3160, 3211, 3340, 3344, 3362, 3377, 3383, 3448, 3492, 3510, 3527, 3551, 3601, 3611, 3620, 3716, 3768, 3782, 3805, 3831, 3851, 3875, 3900, 3906, 3910, 3917, 3967, 3986, 4032, 4046, 4049, 4122, 4145, 4204, 4264, 4282, 4301, 4347, 4362, 4454, 4467, 4476, 4485], '3-hydroxy-3-methylglutaryl-coenzyme': [26, 174, 299, 515], 'A': [27, 175, 300, 322, 516, 994, 2155, 2184, 2218, 2298, 2325, 2830, 2965, 3515, 3949, 3989, 4014, 4076, 4091, 4140, 4158], 'reductase': [28, 176, 301, 517], '(HMG-R)': [29, 177, 518], 'sterol': [31, 179, 302, 1648], 'regulatory': [32, 180, 303, 1649], 'element': [33, 181, 304, 1650], 'binding': [34, 182, 305, 1651], 'cleavage-activating': [36, 184, 307, 1653], '(SCAP).': [38, 186, 1655], 'To': [39, 187, 2480, 2911, 3506, 3871, 4357], 'aid': [40, 188], 'functional': [42, 190, 3763, 3849], 'characterization': [43, 191], 'NPC1,': [45, 193, 568, 1777, 1881, 2260, 4444], 'model': [47, 195, 1876, 3437, 3486, 3521, 4472], 'NPC1': [49, 57, 76, 126, 197, 205, 224, 274, 695, 1005, 1069, 1199, 1232, 1264, 1269, 1401, 1610, 2057, 2151, 2180, 2268, 2279, 2319, 2476, 2488, 2493, 2496, 2616, 2999, 3306, 3345, 3349, 3439, 3493, 3530, 3538, 3544, 3579, 3584, 3634, 3638, 3660, 3681, 3690, 3718, 3760, 3786, 3791, 3810, 3857, 3926, 4105, 4126, 4316, 4348], 'topology': [50, 198, 3339, 3440, 3804], 'was': [51, 199, 583, 1917, 1927, 1945, 1967, 1995, 2018, 2224, 2241, 2364, 2453, 2465, 2546, 2679, 3100, 3155, 3309, 3532, 3950, 4077, 4088, 4096, 4141, 4168, 4209, 4269, 4273, 4385, 4432], 'evaluated': [52, 200], 'by': [53, 73, 201, 221, 502, 2166, 2226, 2243, 2272, 2330, 2350, 2618, 2681, 2685, 2817, 2860, 3037, 3132, 3215, 3714, 3863, 3922, 3983, 4154, 4336], 'expression': [54, 74, 202, 222, 2250, 2368, 2671, 2996, 3639], 'epitope-tagged': [56, 204, 2615, 3537, 3790], 'proteins': [58, 206, 1519, 2489, 2617, 2678, 3034, 3531, 3539, 3761, 3792, 3927, 4034, 4050], 'investigation': [60, 208], 'epitope': [62, 210, 2673, 4437], 'accessibility': [63, 211, 2674], 'in': [64, 85, 129, 212, 233, 277, 342, 391, 451, 495, 505, 694, 949, 957, 1070, 1404, 1410, 1439, 1448, 1453, 1498, 1505, 1639, 1662, 1724, 1903, 2177, 2196, 2235, 2259, 2419, 2457, 2558, 2575, 2599, 2642, 2799, 2812, 2853, 2880, 2890, 2926, 2980, 3005, 3102, 3157, 3250, 3443, 3456, 3497, 3518, 3542, 3631, 3647, 3669, 3785, 3815, 3844, 4037, 4084, 4110, 4128, 4150, 4178, 4186, 4275, 4327, 4341, 4403, 4413, 4438], 'selectively': [65, 213, 2912], 'permeabilized': [66, 214, 3973, 4039, 4064, 4135, 4439], 'cells.': [67, 215, 3589, 3787, 3838, 4040], 'These': [68, 216, 1898, 3536, 3754, 4198], 'results': [69, 217, 341, 3712, 3755, 4045, 4199, 4254, 4463], 'were': [70, 218, 1938, 1955, 1984, 2008, 2026, 2044, 2050, 2158, 2189, 2265, 2270, 2348, 2409, 2506, 2551, 2570, 2609, 2664, 2725, 2760, 2788, 2797, 2815, 2888, 2918, 2973, 3003, 3035, 3073, 3082, 3203, 3228, 3280, 3346, 3540, 3564, 3580, 3593, 3622, 3773, 3812, 3833, 3885, 3893, 3928, 4006, 4017, 4021, 4035, 4117, 4183, 4368], 'further': [71, 219, 2555, 2660, 2884, 4358, 4448], 'confirmed': [72, 220, 4258, 4416], 'identification': [78, 226], 'glycosylated': [80, 228], 'that': [82, 95, 121, 142, 230, 243, 269, 290, 340, 678, 960, 1049, 1196, 1278, 1400, 1445, 1514, 1861, 1883, 2563, 2623, 2650, 2765, 3488, 3689, 3757, 3856, 3965, 4094, 4138, 4161, 4201, 4218, 4230, 4298, 4420, 4441, 4465], 'are': [83, 231, 930, 947, 1052, 1483, 3629, 3657, 3762, 3823, 4057], 'located': [84, 232, 948], 'lumen': [87, 235, 3499], 'endoplasmic': [90, 238, 309, 3503], 'reticulum.': [91, 239], 'Our': [92, 240], 'data': [93, 119, 241, 267, 1443, 1857, 1899], 'indicate': [94, 242, 3756, 4464], 'this': [96, 143, 244, 291, 1178, 1676, 1909, 4162, 4309, 4342, 4480], 'glycoprotein': [97, 245, 1277], '13': [99, 247], 'transmembrane': [100, 248, 316, 1773, 3314], 'domains,': [101, 249, 943, 3451, 3878], '3': [102, 250, 2634, 3673, 3970, 4332], 'large': [103, 251, 1910, 4276], '4': [105, 253, 2805, 2921, 3012, 4042, 4171, 4286, 4405], 'small': [106, 110, 254, 258, 945], 'luminal': [107, 255, 4009, 4191, 4238, 4251, 4260, 4343, 4452], 'loops,': [108, 112, 256, 260], '6': [109, 257, 2802], 'cytoplasmic': [111, 115, 259, 263, 3474, 4115, 4247], 'tail.': [116, 264], 'Furthermore,': [117, 265], 'our': [118, 266, 4214, 4470], 'show': [120, 268, 3643], 'putative': [123, 271, 1780, 1888, 2334], 'SSD': [124, 272, 1664, 1727, 1863, 1889, 4487], 'oriented': [128, 276], 'same': [131, 279, 2958], 'manner': [132, 280], 'as': [133, 281, 1180, 2281, 2423, 2455, 2518, 2668, 2704, 2993, 3045, 3283, 3981, 4023, 4389, 4445], 'those': [134, 282, 1893], 'HMG-R': [136, 284, 1646, 1725, 1895, 3386], 'SCAP,': [138, 286, 1657], 'providing': [139, 287], 'strong': [140, 288], 'evidence': [141, 289], 'domain': [144, 292, 312, 1636, 2336, 2418, 4222], 'functionally': [146, 294, 940, 1865, 3794], 'important.': [147, 295], 'type': [297, 331], 'C': [298, 332, 3614, 3671, 3694, 3771, 4180, 4195, 4232], 'reticulum': [310, 3504], 'trans-Golgi': [313, 453], 'network': [314, 454], 'Patched': [315, 1788], 'wild-type': [317, 2054], 'polyacrylamide': [318], 'gel': [319, 3041], 'electrophoresis': [320, 3042], 'α-galactosidase': [321], 'fluorescein': [323], 'isothiocyanate': [324, 1978], 'phosphate-buffered': [325, 2576], 'saline': [326, 2577], 'bovine': [327, 1925, 2592], 'serum': [328, 1926, 2593], 'albumin': [329, 2594], '(NPC)1': [333], 'rare': [336], 'autosomal': [337], 'recessive': [338], 'disorder': [339], 'progressive': [343], 'neurodegeneration': [344], 'hepatosplenomegaly,': [346], 'leading': [347], 'death': [349], 'during': [350, 2777], 'early': [351], 'childhood': [352], '(1Pentchev': [353, 467, 1122], 'P.G.': [354, 404, 421, 468, 542, 666, 772, 817, 841, 1090, 1123, 1147, 1319, 1359, 1562, 2134, 3735], 'Vanier': [355, 426, 469, 549, 663, 769, 812, 846, 1124, 1152, 1312, 1555, 2131], 'M.T.': [356, 394, 427, 470, 550, 664, 770, 813, 847, 1011, 1125, 1153, 1313, 1556, 2132], 'Suzuki': [357, 471, 1126], 'M.C.': [358, 360, 472, 474, 660, 766, 1127, 1129, 1315, 1558, 2128], 'Patterson': [359, 473, 659, 765, 1128, 1314, 1557, 2127], 'Scriver': [361, 475, 1130], 'C.R.': [362, 476, 628, 734, 1131, 2096], 'Beaudet': [363, 477, 1132], 'A.L.': [364, 478, 1133], 'Metabolic': [365, 479, 1134], 'Molecular': [367, 481, 1136, 1970, 1988, 2296, 3224], 'Basis': [368, 482, 1137], 'Inherited': [370, 484, 1139], 'Disease.': [371, 485, 1140], 'McGraw-Hill,': [372, 486, 1141], 'New': [373, 487, 1142, 1958], 'York1995:': [374, 488, 1143], '2625-2639Google': [375, 489, 1144], 'Scholar).': [376, 446, 490, 566, 993, 1257, 1395, 1433, 1607, 1771, 1854, 2145, 2310, 2539, 3065, 3276, 3303, 3753], 'most': [378, 929, 1223], 'prominent': [379], 'biochemical': [380], 'feature': [381], 'accumulation': [384, 1447, 3784], 'low': [386, 521, 3915], 'density': [387, 522], 'lipoprotein-derived': [388], 'unesterified': [389], 'cholesterol': [390, 449, 496, 513, 1186, 1446, 1644, 2612, 3702, 3783, 4324], 'lysosomes': [392, 499, 1286, 1411, 3997], '(2Vanier': [393], 'Rodriguez-Lafrasse': [395, 1014], 'C.': [396, 602, 708, 833, 1015, 1239, 2070], 'Rousson': [397], 'R.': [398, 647, 753, 2115], 'Gazzah': [399], 'N.': [400, 809], 'Juge': [401], 'M.-C.': [402], 'Pentchev': [403, 665, 771, 816, 840, 1016, 1318, 1358, 1561, 2133, 3734], 'Revol': [405], 'A.': [406, 624, 626, 730, 732, 807, 1347, 1368, 1681, 1701, 1714, 1834, 1845, 2092, 2094, 3404, 3745], 'Louisot': [407], 'P.': [408, 1017], 'Biochim.': [409, 436, 1162], 'Biophys.': [410, 437, 1163], 'Acta.': [411, 438, 1164], '1991;': [412], '1096:': [413], '328-337Crossref': [414], 'PubMed': [415, 442, 538, 564, 673, 779, 825, 860, 889, 922, 989, 1042, 1086, 1114, 1168, 1219, 1253, 1333, 1372, 1391, 1429, 1477, 1576, 1603, 1695, 1718, 1751, 1769, 1812, 1850, 2141, 2385, 2535, 2720, 2848, 3061, 3272, 3299, 3400, 3420, 3749], 'Scopus': [416, 443, 674, 780, 826, 861, 890, 923, 990, 1043, 1115, 1169, 1254, 1334, 1373, 1392, 1430, 1478, 1577, 1604, 1696, 1719, 1752, 1813, 1851, 2142, 2386, 2536, 2721, 2849, 3062, 3273, 3300, 3421, 3750], '(193)': [417], 'Google': [418, 445, 539, 565, 676, 782, 828, 863, 892, 925, 992, 1027, 1045, 1087, 1117, 1171, 1220, 1256, 1336, 1375, 1394, 1432, 1480, 1579, 1606, 1698, 1721, 1754, 1770, 1815, 1853, 2144, 2388, 2538, 2723, 2851, 3064, 3275, 3302, 3401, 3423, 3752], 'Scholar,': [419, 540, 783, 829, 864, 893, 1028, 1088, 1145, 1337, 1376, 1580, 1699, 1755, 1816, 3402], '3Pentchev': [420, 1146], 'Brady': [422, 661, 767, 1148, 1316, 1354, 1559, 2129, 3732], 'R.O.': [423, 662, 768, 1149, 1317, 1355, 1560, 2130, 3733], 'Blanchette-Mackie': [424, 629, 735, 1150, 1320, 1348, 1563, 2097, 3722], 'E.J.': [425, 630, 736, 1151, 1321, 1349, 1564, 2098, 3723], 'Carstea': [428, 1018, 1154, 1305, 1548], 'E.D.': [429, 592, 698, 1019, 1155, 1306, 1549, 2060], 'Parker': [430, 1156], 'C.C.': [431, 1157], 'Goldin': [432, 1158], 'E.': [433, 787, 1159], 'Roff': [434, 1160, 1299, 1542], 'C.F.': [435, 1161, 1300, 1543], '1994;': [439, 1165, 1213], '1225:': [440, 1166], '235-243Crossref': [441, 1167], '(107)': [444, 1170], 'In': [447, 491, 1188, 1490, 1656, 1867, 3483, 4062, 4173, 4479], 'addition,': [448, 1189, 1491, 3484], 'accumulates': [450], '(TGN),': [455], 'its': [457, 1663, 3993, 4259, 4267, 4436], 'relocation': [458], 'from': [461, 498, 1522, 1626, 1919, 1928, 1940, 1947, 1957, 1968, 1986, 1996, 2009, 2020, 2046, 2314, 2500, 2694, 3458, 4051, 4245, 4469], 'plasma': [463, 2915, 3912, 3975, 4100, 4280, 4351, 4399], 'membrane': [464, 1276, 1911, 3153, 3803, 3880, 3913, 3976, 4281, 4290, 4352], 'delayed': [466], 'fibroblasts,': [492], 'defect': [494], 'exit': [497], 'accompanied': [501, 4153], 'an': [503, 587, 1181, 1436, 2168, 2353, 4371, 4426], 'attenuation': [504], 'down-regulation': [507], 'two': [509, 1640, 4365], 'key': [510, 1641], 'components': [511], 'homeostasis,': [514, 1645], 'lipoprotein': [523], 'receptor': [524, 1791], '(4Liscum': [525, 1073], 'L.': [526, 638, 744, 1074, 1209, 2106], 'Faust': [527, 1075, 1736], 'J.R.': [528, 1076, 1737], 'J.': [529, 555, 604, 612, 649, 710, 718, 755, 849, 878, 911, 978, 1021, 1035, 1077, 1098, 1100, 1110, 1210, 1242, 1322, 1470, 1565, 1596, 1760, 2072, 2080, 2117, 2291, 2844, 3057, 3295, 3391, 3409], 'Biol.': [530, 556, 1078, 1243, 1323, 1473, 1566, 1599, 1761, 3327, 3392, 3410], 'Chem.': [531, 557, 1079, 1244, 1324, 1567, 1762, 3328, 3393, 3411], '1987;': [532, 1080, 1111], '262:': [533, 1081], '17002-17008Abstract': [534, 1082], 'Full': [535, 561, 855, 857, 884, 886, 917, 919, 984, 986, 1083, 1216, 1248, 1250, 1328, 1330, 1571, 1573, 1690, 1692, 1748, 1766, 3397, 3415, 3417], 'Text': [536, 562, 856, 858, 885, 887, 918, 920, 985, 987, 1084, 1217, 1249, 1251, 1329, 1331, 1572, 1574, 1691, 1693, 1749, 1767, 3398, 3416, 3418], 'PDF': [537, 563, 859, 888, 921, 988, 1085, 1218, 1252, 1332, 1575, 1694, 1750, 1768, 3399, 3419], '5Pentchev': [541], 'Kruth': [543, 1093], 'H.S.': [544, 1094], 'Comly': [545, 621, 727, 1091, 2089], 'M.E.': [546, 620, 726, 1092, 1378, 1416, 2088, 2372, 2522, 2707, 2840, 3053, 3291], 'Butler': [547, 1097], 'J.D.': [548], 'Wenger': [551, 844], 'D.A.': [552, 668, 774, 845, 2136], 'Patel': [553, 1107, 1291, 1352, 1356, 1534, 3728], 'S.': [554, 797, 805, 1013, 1108, 1292, 1294, 1341, 1367, 1462, 1535, 1537, 1588, 1713, 3729, 3744], '1986;': [558], '261:': [559], '16775-16780Abstract': [560], 'Recently': [567], 'gene': [570, 934, 1179, 1233, 2738], 'for': [571, 1054, 1230, 1486, 1792, 1877, 2337, 2344, 2403, 2553, 2579, 2605, 2611, 2630, 2656, 2729, 2801, 2856, 2923, 2968, 2983, 3078, 3147, 3188, 3197, 3205, 3255, 3438, 3582, 3595, 3616, 3626, 3637, 3659, 3943, 4059, 4071, 4098, 4255], 'major': [573, 3512], 'linkage': [574], 'complementation': [576, 1008], 'group,': [577], 'which': [578, 1059, 1482, 1966, 2458, 3617, 3656, 3665, 4337], 'maps': [579], 'chromosome': [581], '18q11-12,': [582], 'identified.': [584], 'It': [585], 'encodes': [586], 'approximately': [588, 2756], '4.5-kilobase': [589], 'mRNA': [590], '(6Carstea': [591, 697, 2059], 'Morris': [593, 699, 814, 838, 1303, 1546, 2061], 'J.A.': [594, 700, 815, 839, 1304, 1547, 2062], 'Coleman': [595, 701, 2063], 'K.G.': [596, 702, 2064], 'Loftus': [597, 703, 2065], 'S.K.': [598, 704, 2066], 'Zhang': [599, 705, 794, 2067], 'D.': [600, 651, 706, 757, 1032, 1832, 2068, 2119], 'Cummings': [601, 707, 2069], 'Gu': [603, 709, 1825, 2071], 'Rosenfeld': [605, 711, 2073], 'M.A.': [606, 712, 835, 1207, 2074], 'Pavan': [607, 713, 2075], 'W.J.': [608, 714, 2076], 'Krizman': [609, 715, 2077], 'D.B.': [610, 716, 2078], 'Nagle': [611, 717, 2079], 'Polymeropoulos': [613, 719, 2081], 'M.H.': [614, 720, 2082], 'Sturley': [615, 721, 2083], 'S.L.': [616, 722, 2084], 'Ioannou': [617, 723, 1383, 1421, 2085, 2377, 2527, 2712], 'Y.A.': [618, 724, 1384, 1422, 2086, 2378, 2528, 2713, 2836, 3049, 3287], 'Higgins': [619, 725, 2087], 'M.': [622, 645, 658, 728, 751, 764, 793, 1102, 1290, 1460, 1533, 1586, 1820, 1822, 1838, 2090, 2113, 2126], 'Cooney': [623, 729, 1295, 1346, 1538, 2091], 'Brown': [625, 731, 1684, 1704, 1742, 2093, 3405], 'Kaneski': [627, 733, 2095], 'Dwyer': [631, 737, 1297, 1540, 2099, 3724], 'N.K.': [632, 738, 1205, 1298, 1541, 2100, 3725], 'Neufeld': [633, 739, 1350, 2101, 3730], 'E.B.': [634, 740, 1288, 1351, 1531, 2102, 3731], 'Chang': [635, 741, 1240, 2103], 'T.Y.': [636, 742, 1241, 2104], 'Liscum': [637, 743, 1208, 2105], 'Straus': [639, 745, 2107], 'III,': [640, 746, 1310, 1553, 2108, 3737], 'J.F.': [641, 747, 1311, 1554, 2109, 3738], 'Ohno': [642, 748, 818, 842, 1301, 1544, 2110], 'K.': [643, 749, 791, 803, 819, 843, 1302, 1545, 2111, 3324], 'Zeigler': [644, 750, 2112], 'Carmi': [646, 752, 2114], 'Sokol': [648, 754, 1099, 2116], 'Marhie': [650, 756, 2118], "O'Neill": [652, 758, 2120], 'R.R.': [653, 759, 2121], 'van': [654, 760, 2122], 'Diggelen': [655, 761, 2123], 'O.P.': [656, 762, 2124], 'Ellender': [657, 763, 2125], 'Tagle': [667, 773, 2135], 'Science.': [669, 775, 2137], '1997;': [670, 776, 2138], '277:': [671, 777, 2139], '228-231Crossref': [672, 778, 2140], '(1230)': [675, 781, 2143], 'Scholar)': [677, 1046, 1118, 1172, 1221, 2389, 2852, 3332, 3424], 'produce': [682], '1278-amino': [684], 'acid': [685, 2207, 3473], 'protein.': [686], 'At': [687, 2540, 2782, 3887], 'least': [688, 2332], '36': [689, 3068], 'mutations': [690], 'have': [691, 1047, 1173, 1201, 1224, 1398, 3354], 'been': [692, 1174, 1202, 1225, 1271, 2767], 'described': [693, 2519, 2705, 3046, 3284], 'patients': [696, 999, 1051], '7Yamamoto': [784], 'T.': [785, 801, 837, 1096, 1456, 1582, 2295], 'Nanba': [786], 'Ninomiya': [788], 'H.': [789, 795, 1466, 1592, 1836, 3721], 'Higaki': [790], 'Taniguchi': [792], 'Akaboshi': [796], 'Watanabe': [798], 'Y.': [799, 1803], 'Takeshima': [800], 'Inui': [802], 'Okada': [804], 'Tanaka': [806], 'Sakuragawa': [808], 'Millat': [810], 'G.': [811, 831, 1735], 'Hum.': [820, 850, 879, 912, 979, 1022], 'Genet.': [821, 851, 880, 913, 980, 1023, 1386, 1424, 2380, 2530, 2715], '1999;': [822, 852, 881, 981, 1325, 1369, 1388, 1426, 1474, 1568, 1600, 2382, 2532, 2717, 3746], '105:': [823], '10-16Crossref': [824], '(71)': [827], '8Millat': [830], 'Marcais': [832], 'Rafi': [834], 'Yamamoto': [836], 'Am.': [848, 877, 910, 977, 1020], '65:': [853, 882, 982], '1321-1329Abstract': [854], '(152)': [862, 1255], '9Greer': [865], 'W.L.': [866, 895, 966], 'Dobson': [867, 906, 967], 'M.J.': [868, 907, 968], 'Girouard': [869, 900, 969], 'G.S.': [870, 901, 970], 'Byers': [871, 904, 971], 'D.M.': [872, 905, 972, 1818], 'Riddell': [873, 896, 973], 'D.C.': [874, 897, 974, 1106], 'Neumann': [875, 908, 975], 'P.E.': [876, 909, 976], '1252-1260Abstract': [883, 983], '(89)': [891, 991], '10Greer': [894], 'Gillan': [898], 'T.L.': [899], 'Sparrow': [902], 'S.M.': [903], '1998;': [914, 2845, 3058, 3296, 3412], '63:': [915], '52-54Abstract': [916], '(83)': [924, 2850, 3063, 3301], 'Scholar),': [926, 1481, 1722, 2724], 'although': [928, 1609], 'found': [931, 1638], 'throughout': [932], 'do': [936], 'not': [937, 1001, 1612, 3676, 3697, 3869, 3978, 4132, 4224, 4312, 4395, 4458], 'reveal': [938], 'any': [939, 1616], 'critical': [941], 'cluster': [946, 1620], 'carboxyl-terminal': [952], 'third': [953], 'possesses': [961], 'conserved': [962], 'cysteine': [963], 'residues': [964, 1627], '(9Greer': [965], 'minor': [995], 'group': [996], 'NPC': [998, 1449, 1492], 'does': [1000, 3696, 4223, 4311], 'map': [1002], 'locus,': [1006], 'studies': [1009, 1397, 1869, 3840, 3963], '(11Vanier': [1010], 'Duthel': [1012], '1996;': [1024, 1039, 1687, 1715, 1809, 1847], '58:': [1025], '118-125PubMed': [1026], '12Steinberg': [1029], 'S.J.': [1030], 'Mondal': [1031], 'Fensom': [1033], 'A.H.': [1034], 'Inherit.': [1036], 'Metab.': [1037, 1387, 1425, 2381, 2531, 2716], 'Dis.': [1038], '19:': [1040], '769-774Crossref': [1041], '(29)': [1044], 'shown': [1048, 1226, 1272, 3442, 3630, 4058, 4402], 'these': [1050, 1856, 1868, 2187, 2433, 3430, 3789, 3837, 4001], 'defective': [1053, 1229, 1497], 'second': [1056, 2219], 'gene,': [1057], 'NPC2,': [1058], 'currently': [1061, 4354], 'unknown.': [1062, 1268], 'effects': [1064], 'reduced': [1066], 'or': [1067, 2951, 3316, 3865, 3919], 'absent': [1068], 'both': [1071, 4184], 'humans': [1072], '13Pentchev': [1089], 'Tokoro': [1095], 'Filling-Katz': [1101], 'Quirk': [1103], 'J.M.': [1104, 1805, 3727], 'Marshall': [1105], 'FASEB': [1109], '1:': [1112, 1475, 1601], '40-45Crossref': [1113], '(149)': [1116], 'animal': [1120], 'models': [1121], 'well': [1175], 'studied,': [1176], 'establishing': [1177], 'essential': [1182], 'component': [1183], 'intracellular': [1185, 3979], 'trafficking.': [1187], 'several': [1190], 'Chinese': [1191], 'hamster': [1192], 'ovary': [1193], 'cell': [1194, 2502, 2775, 3098, 3152, 4306], 'mutants': [1195], 'display': [1197], 'phenotype': [1200], 'characterized': [1203], '(14Dahl': [1204], 'Daunais': [1206], 'Lipid': [1211], 'Res.': [1212], '35:': [1214], '1839-1849Abstract': [1215], '(15Cruz': [1234], 'J.C.': [1235], 'Sugii': [1236], 'S.,': [1237], 'Yu,': [1238], '2000;': [1245], '275:': [1246], '4013-4021Abstract': [1247], 'However,': [1258, 4083, 4266, 4308], 'precise': [1260, 1906], 'function(s)': [1261], 'still': [1267], 'has': [1270], 'localizes': [1279], 'Lamp-positive': [1281], 'organelles,': [1282], 'presumably': [1283], 'endosomes': [1284, 1406, 1454, 1524], '(16Neufeld': [1287, 1530], 'Wastney': [1289, 1532], 'Suresh': [1293, 1340, 1536], 'A.M.': [1296, 1539], 'Incardona': [1307, 1550], 'J.P.': [1308, 1380, 1418, 1551, 2374, 2524, 2709], 'Strauss': [1309, 1552, 3736], '274:': [1326, 1569], '9627-9635Abstract': [1327, 1570], '(334)': [1335, 1578], '17Patel': [1338], 'S.C.': [1339], 'Kumar': [1342], 'U.': [1343, 1366, 1712, 3743], 'Hu': [1344], 'C.Y.': [1345], 'R.C.': [1353], 'Y.C.': [1357], 'Ong': [1360], 'W.Y.': [1361], 'Proc.': [1362, 1708, 3739], 'Natl.': [1363, 1709, 3740], 'Acad.': [1364, 1710, 3741], 'Sci.': [1365, 1711, 3742], '96:': [1370, 3747], '1657-1662Crossref': [1371], '(146)': [1374], '18Higgins': [1377], 'Davies': [1379, 1417, 2373, 2523, 2708], 'Chen': [1381, 1419, 2375, 2525, 2710], 'F.W.': [1382, 1420, 2376, 2526, 2711], 'Mol.': [1385, 1423, 2379, 2529, 2714], '68:': [1389, 1427, 2383, 2533, 2718], '1-13Crossref': [1390, 1428, 2384, 2534, 2719], '(214)': [1393, 1431, 2387, 2537, 2722], 'Further': [1396], 'demonstrated': [1399, 3982], 'resides': [1402], 'primarily': [1403, 1452], 'late': [1405, 1523], 'only': [1408], 'secondarily': [1409], 'TGN': [1414, 1527], '(18Higgins': [1415, 2371, 2521, 2706], 'This': [1434, 2472, 4228, 4271], 'important': [1437], 'distinction': [1438], 'view': [1440, 1860], 'recent': [1442], 'suggesting': [1444, 1513, 4189], 'cells': [1450, 1493, 2550, 2569, 2663, 2759, 2787, 2796, 2887, 2917, 2972, 3002, 3072, 3563, 3575, 3592, 3603, 3621, 3635, 3817, 3822, 3867, 3892, 4020, 4056, 4086, 4136, 4284, 4415, 4440], 'occurs': [1451], '(19Kobayashi': [1455], 'Beuchat': [1457, 1583], 'M.-H.': [1458, 1584], 'Lindsay': [1459, 1585], 'Frias': [1461, 1587], 'Palmiter': [1463, 1589], 'R.D.': [1464, 1590, 3390], 'Sakuraba': [1465, 1591], 'Parton': [1467, 1593], 'R.G.': [1468, 1594], 'Gruenberg': [1469, 1595], 'Nat.': [1471, 1597], 'Cell': [1472, 1598], '113-118Crossref': [1476, 1602], '(251)': [1479, 1605], 'sorting': [1484, 1507], 'sites': [1485, 2188], 'various': [1487, 2998], 'cellular': [1488], 'components.': [1489], 'appear': [1494, 3677], 'their': [1499, 2420, 3370, 3374, 3556, 4190], 'efflux': [1500], 'endocytosed': [1502], 'sucrose': [1503, 2927], 'mannose-6': [1510], 'phosphate': [1511], 'receptor,': [1512], 'retrograde': [1516], 'movement': [1517], 'cargo': [1521], 'perturbed': [1529], '19Kobayashi': [1581], 'Interestingly,': [1608, 4288], 'closely': [1613], 'related': [1614], 'known': [1617], 'proteins,': [1618, 3854, 4106], 'five': [1622], 'potential': [1623, 3313, 3478], 'membrane-spanning': [1624, 2335], 'sequences,': [1625], '616': [1628], '797,': [1630], 'homologous': [1632], 'regulators': [1642], 'single': [1659, 4237], 'point': [1660], 'mutation': [1661], 'sufficient': [1666], 'render': [1668], 'sterol-insensitive,': [1671], 'indicating': [1672, 3855, 4079, 4297], 'importance': [1674], '(20Hua': [1678], 'X.': [1679, 1703], 'Nohturfft': [1680], 'Goldstein': [1682, 1706, 1740, 3407], 'J.L.': [1683, 1707, 1741, 3408], 'M.S.': [1685, 1705, 1743, 3406], 'Cell.': [1686, 1744], '87:': [1688], '415-426Abstract': [1689], '(427)': [1697], '21Nohturfft': [1700], 'Hua': [1702], '93:': [1716], '13709-13714Crossref': [1717], '(57)': [1720], 'whereas': [1723], 'mediates': [1728], 'sterol-regulated': [1729], 'degradation': [1730], '(22Gil': [1734], 'Chin': [1738], 'D.J.': [1739], '1985;': [1745, 1763], '41:': [1746], '249-258Abstract': [1747], '(259)': [1753], '23Luskey': [1756], 'K.L.': [1757], 'Stevens': [1758], 'B.': [1759], '260:': [1764], '10271-10277Abstract': [1765], '(TM)': [1774, 3315], 'regions': [1775, 1786, 3357, 3365], 'including': [1778, 4011], 'SSD,': [1781], 'also': [1782, 1985], 'share': [1783], '(PTC),': [1789], 'cholesterol-activated': [1794], 'sonic': [1796], 'hedgehog': [1797], '(24Marigo': [1798], 'V.': [1799], 'Davey': [1800], 'R.A.': [1801], 'Zuo': [1802], 'Cunningham': [1804], 'Tabin': [1806], 'C.J.': [1807], 'Nature.': [1808, 1846, 3268], '384:': [1810, 1848], '176-179Crossref': [1811], '(719)': [1814], '25Stone': [1817], 'Hynes': [1819], 'Armanini': [1821], 'Swanson': [1823], 'T.A.': [1824], 'Q.': [1826], 'Johnson': [1827], 'R.L.': [1828], 'Scott': [1829], 'M.P.': [1830], 'Pennica': [1831], 'Goddard': [1833], 'Phillips': [1835], 'Noll': [1837], 'Hooper': [1839], 'J.E.': [1840], 'de': [1841], 'Sauvage': [1842], 'F.': [1843], 'Rosenthal': [1844], '129-133Crossref': [1849], '(971)': [1852], 'Together': [1855], 'support': [1858, 1901, 4449], 'significant.': [1866], 'we': [1870], 'describe': [1871], 'validate': [1873, 3507], 'topological': [1875, 4471], 'structure': [1879], 'showing': [1882], 'orientation': [1885, 4203, 4475], 'identical': [1891, 3860], 'SCAP.': [1897], 'should': [1900], 'efforts': [1902], 'elucidating': [1904], 'function': [1907], 'glycoprotein.': [1912], "Dulbecco's": [1913, 2559], 'modified': [1914, 2560], "Eagle's": [1915, 2561], 'medium': [1916, 2545, 2562], 'purchased': [1918, 1946, 2019], 'Mediatech': [1920], '(Herndon,': [1921], 'VA)': [1922, 2701], 'fetal': [1924], 'HyClone': [1929], '(Logan,': [1930], 'UT).': [1931], 'LipofectAMINE,': [1932, 3019], 'l-glutamine,': [1933], 'gentamicin,': [1934], 'colcemid,': [1935], 'trypsin/EDTA': [1937], 'obtained': [1939, 1956, 2045, 2499, 2693, 3713], 'Life': [1941], 'Technologies,': [1942], 'Inc.': [1943, 2013, 2022], 'Fluoromount-G': [1944], 'Southern': [1948], 'Biotechnology': [1949], 'Associates': [1950], '(Birmingham,': [1951], 'AL).': [1952], 'All': [1953, 2041, 2048, 2346, 3925], 'enzymes': [1954], 'England': [1959], 'BioLabs': [1960], '(Beverly,': [1961], 'MA),': [1962], 'except': [1963], 'forN-glycosidase': [1964], 'F,': [1965, 4406], 'Roche': [1969, 1987], 'Biochemicals': [1971], '(Indianapolis,': [1972], 'IN).': [1973], 'goat': [1975, 1981, 2002], 'anti-mouse': [1976, 2003, 2654, 2871], 'IgG-fluorescein': [1977], '(FITC)': [1979], 'anti-rabbit': [1982, 2006, 2876], 'IgG-FITC': [1983, 2872], 'Biochemicals,': [1989], 'anti-FLAG': [1991, 2628, 2823, 3212, 3570], 'M2': [1992, 2627, 2822, 2961, 3932], 'monoclonal': [1993], 'antibody': [1994, 2629, 2824, 2834, 3571, 3934, 3956, 3990, 4081, 4427], 'Eastman': [1997], 'Kodak': [1998], 'Co.': [1999], 'IgG-rhodamine-X': [2004, 2007, 2655, 2877], 'Jackson': [2010], 'Immunoresearch': [2011], 'Laboratories': [2012], '(West': [2014], 'Grove,': [2015], 'PA).': [2016, 2024], 'Filipin': [2017, 2641], 'Polysciences,': [2021], '(Warrington,': [2023], 'Oligonucleotides': [2025], 'synthesized': [2027], 'using': [2028, 2052, 2276, 2287, 2514, 2735, 2895, 3011, 3084, 3134, 3178, 3836, 3914, 3952], 'phosphoramidite': [2029], 'chemistry': [2030], 'on': [2031, 2352, 2762, 3369, 3429], '380B': [2033], 'DNA': [2034, 2358, 2754], 'synthesizer': [2035], '(Applied': [2036], 'Biosystems,': [2037], 'Foster': [2038], 'City,': [2039], 'CA).': [2040], 'other': [2042], 'reagents': [2043], 'Sigma-Aldrich.': [2047], 'plasmids': [2049], 'constructed': [2051, 3533], '(wt)': [2055], 'human': [2056, 2495, 3543, 3633, 3864, 4329], 'cDNA': [2058, 2280, 2312, 2363, 2421], 'For': [2146, 2182, 2253, 2793, 2995, 3597], 'analysis': [2147, 2254, 3265, 3897], 'full-length': [2150, 2486, 3529, 3808, 4104], 'protein,': [2152, 4016], 'hydrophilic': [2153, 3317, 3342, 3453, 3513, 3877], 'loops': [2154, 2183, 2324, 3343, 3454, 3514, 4179, 4205, 4231], 'E': [2157, 2221, 2327, 3517, 4208, 4242, 4257, 4384, 4422, 4431, 4456], 'tagged': [2159], 'FLAG': [2162, 2229, 2470, 2962, 3933, 4108, 4176, 4339, 4376], 'peptide': [2163], 'sequence': [2164, 2230, 2237, 2401, 3308], 'DYKDDDDK': [2165], 'inserting': [2167], 'adapter': [2169], 'oligonucleotide': [2170], 'at': [2171, 2203, 2231, 2331, 2390, 2742, 2804, 2920, 3067, 3091, 3143, 3193, 3258, 3333, 4278, 4304, 4378], 'unique': [2173], 'restriction': [2174], 'endonuclease': [2175], 'site': [2176, 2233, 2343, 3781], 'wt': [2179, 2278, 4443], 'cDNA.': [2181], 'E,': [2186, 4364], 'NdeI,ClaI,': [2190], 'BsiWI,': [2191], 'BclI,': [2192], 'andBsu36I': [2193], 'resulted': [2195], 'insertion': [2198], 'following': [2201, 3818], 'sequences': [2202, 2422], 'indicated': [2205, 3964, 4137, 4160, 4200], 'amino': [2206, 2435, 2477, 3460, 3466, 3490, 4483], 'positions:': [2208], '36-FLAG-Y,': [2209], '306-FLAG-I,': [2210], '519-FLAG-VR,': [2211], '554-FLAG-DQ,': [2212], '992-FLAG-DQ,': [2214], 'respectively': [2215, 2444], '(Fig.1': [2216, 3358, 3425], 'B).': [2217, 4172], 'loop': [2220, 2415, 3613, 3670, 3693, 3770, 4124, 4139, 4147, 4166, 4239, 4241, 4256, 4344, 4363, 4383, 4421, 4430, 4455], 'tag': [2222, 2240, 3668, 3695, 4340, 4377], 'construct': [2223, 3615, 3628, 4373], 'created': [2225], 'placing': [2227], 'theSphI': [2232], 'resulting': [2234], '886-FLAG-HA.': [2238], 'Each': [2239, 2311, 2361], 'verified': [2242, 2349], 'sequencing': [2244, 2351], 'prior': [2245, 2732, 3185], 'cloning': [2247], 'into': [2248, 2366, 2755], 'vector': [2251, 2369], 'pAsc9.': [2252], 'glycosylation': [2257], 'pattern': [2258, 4070], 'number': [2262], 'constructs': [2264, 2408, 2513, 3599, 3811], 'created.': [2266], 'Truncated': [2267], 'cDNAs': [2269, 2347], 'generated': [2271], 'polymerase': [2273], 'chain': [2274], 'reaction': [2275], 'template': [2283], 'then': [2285, 2586, 2645, 2866, 2974], 'cloned': [2286, 2365], 'standard': [2288], 'procedures': [2289], '(26Sambrook': [2290], 'Fritsch': [2292], 'E.F.': [2293], 'Maniatis': [2294], 'Cloning:': [2297], 'Laboratory': [2299, 2304], 'Manual.': [2300], 'Cold': [2301, 2306], 'Spring': [2302, 2307], 'Harbor': [2303], 'Press,': [2305], 'Harbor,': [2308], 'NY1989Google': [2309], 'extended': [2313], 'start': [2316], 'codon': [2317], '3′-end': [2322], '(Fig.2),': [2328], 'followed': [2329, 2684, 2859, 3214], 'one': [2333, 3663, 3899], 'correct': [2338, 2491, 3557], 'anchorage': [2340], 'aBamHI': [2342], 'cloning.': [2345], 'automated': [2354], 'ABI': [2355], 'prism': [2356], '377': [2357], 'sequencer': [2359], '(Perkin-Elmer).': [2360], 'truncated': [2362], 'pAsc9': [2370], 'BamHI': [2392], 'site,': [2393], 'adjacent': [2394], '3′': [2397], 'in-frame': [2398], 'FLAG-tag': [2399], 'linker': [2400], '(DYKDDDDK-stop)': [2402], 'convenient': [2404], 'detection.': [2405], 'plasmid': [2407, 2753], 'named': [2410], 'according': [2411, 3026], 'last': [2414], 'TM': [2417, 3336, 3356, 3364, 3450], 'follows:': [2424], 'pLaTM1,': [2425], 'pLbTM2,': [2426], 'pLcTM3,': [2427], 'pLdTM4,': [2428], 'pLdTM6,': [2429], 'pLeTM13;': [2431], 'encoded': [2434, 2475], 'acids': [2436, 2478, 3461, 3467], '1–296,': [2437], '375,': [2438], '549,': [2439], '644,': [2440], '727,': [2441], '1164,': [2443], '(Fig.': [2445, 3522, 3672, 4119, 4170, 4193, 4285, 4331], '2).': [2446], 'An': [2447], 'additional': [2448, 4372], 'slightly': [2449], 'truncated,': [2450], 'FLAG-tagged': [2451, 2487, 2677, 3578, 3759, 3809, 4053, 4411], 'produced,': [2454], 'above,': [2456, 4446], 'carboxyl': [2460], 'terminus': [2461, 3491, 4484], 'tail': [2462, 3475, 3719], 'after': [2463], 'TM15': [2464], 'completely': [2466], 'replaced': [2467, 2547], 'tag.': [2471], 'plasmid,': [2473], 'pTM15tail,': [2474], '1–1252.': [2479], 'assess': [2481], 'ability': [2483], 'phenotype,': [2494, 3585], 'fibroblasts': [2497, 3545, 4330], '(GM03123),': [2498], 'Coriell': [2501], 'repositories': [2503], '(Camden,': [2504], 'NJ),': [2505], 'grown': [2507, 2552, 2703, 2761, 3004], 'transfected': [2509, 3010], 'appropriate': [2512], 'LipofectAMINE': [2515], 'Plus': [2516, 3024], 'reagent': [2517], 'previously': [2520, 3047, 3285], '24': [2541, 2556, 2783, 3888], 'h': [2542, 2557, 2731, 2784, 3069, 3207, 3889], 'post-transfection,': [2543, 2785, 3070, 3890], 'contained': [2564, 2624, 2651, 3666], '5%': [2565], 'lipoprotein-deficient': [2566], 'serum.': [2567], 'fixed': [2571, 2798, 2975], '3%': [2573], 'paraformaldehyde': [2574], '(PBS)': [2578], '30': [2580, 2984, 3021], 'min,': [2581, 2632, 2858], 'washed': [2582, 2789, 3229], 'PBS,': [2584, 2643, 2864, 3085], 'quenched': [2587], 'blocking': [2589, 2621, 2648, 2854, 2881, 2981], 'solution': [2590, 2622, 2649, 2855, 2982], '(3%': [2591], '(BSA),': [2595], '50': [2596, 2639, 3107, 3164], 'μg/ml': [2597, 2626, 2640, 2653, 2821, 2827, 2870, 2875, 2953, 3115, 3118, 3210, 3971], 'filipin': [2598, 3567, 3648], 'PBS)': [2600], 'containing': [2601, 2947, 3476, 3691, 4107, 4374], '1.5': [2602], 'mg/ml': [2603, 2771], 'glycine': [2604], '15': [2606, 3189, 3355, 3449], 'min.': [2607, 2658, 3096, 3149, 3199], 'Cells': [2608, 3081], 'costained': [2610, 3565], 'incubating': [2619, 2646], '2': [2625, 2652, 2869, 2874, 2952, 3086, 3112, 3167], '45': [2631, 2657, 2857, 2924], 'washing': [2633, 2661], '×': [2635, 3093, 3145, 3195], '5': [2636, 3117, 3452], 'min': [2637, 2803, 2925, 2985, 3190, 3257], 'After': [2659, 2883], 'mounted': [2665, 2889], 'visualized': [2667, 2894], 'above.': [2669, 2994, 4390], 'all': [2676, 3598], 'assessed': [2680], 'transient': [2682], 'transfection': [2683, 4028], 'selective': [2686, 2779, 3879, 3908, 4097], 'permeabilization': [2687, 2780, 3881, 3904, 3909, 3921, 4095], 'immunofluorescence': [2689, 3883, 3896, 4047], 'microscopy.': [2690], 'COS-7': [2691, 3001, 3816, 3821], 'cells,': [2692, 3655, 4065, 4152, 4188], 'American': [2696], 'Type': [2697], 'Culture': [2698], 'Collection': [2699], '(Manassas,': [2700], 'treated': [2726, 2768], 'colcemid': [2728], '18': [2730], 'electroporation': [2734], 'Bio-Rad': [2737], 'pulser': [2739], 'unit': [2740], 'set': [2741], '500': [2743], 'microfarads': [2744], '250': [2746], 'V': [2747], 'transfect': [2749, 3826], '10': [2750, 2942, 3095, 3114, 3148, 3179, 3198, 3256], 'μg': [2751, 3013], '106cells.': [2757], 'glass': [2763], 'coverslips': [2764], 'had': [2766], '0.1': [2770, 2933, 3128], 'poly-l-lysine': [2772], 'reduce': [2774], 'loss': [2776], 'subsequent': [2778], 'steps.': [2781], 'twice': [2790], 'PBS.': [2792, 2813], 'complete': [2794, 2969, 4080], 'permeabilization,': [2795, 3944], 'methanol': [2800, 2977, 3923], '°C': [2806, 2922], 'blocked': [2808], '1%': [2810, 3170], 'BSA': [2811], 'Proteins': [2814], 'detected': [2816, 3929, 3951, 4007, 4274], 'incubation': [2818, 2867, 3218], '5.2': [2820], '1.4': [2826], 'rabbit': [2828], 'anti-α-galactosidase': [2829], '(α-Gal': [2831], 'A)': [2832, 3495], 'polyclonal': [2833, 3955], '(27Ioannou': [2835, 3048, 3286], 'Zeidner': [2837, 3050, 3288], 'K.M.': [2838, 3051, 3289], 'Grace': [2839, 3052, 3290], 'Desnick': [2841, 3054, 3292], 'R.J.': [2842, 3055, 3293], 'Biochem.': [2843, 3056, 3294], '332:': [2846, 3059, 3297], '789-797Crossref': [2847, 3060, 3298], 'wash': [2862], 'secondary': [2878, 2989, 3938, 3960], 'antibodies': [2879, 2966, 2990], 'solution.': [2882], 'washing,': [2885], 'Fluoromount': [2891], 'G': [2892], 'Nikon': [2897], 'Eclipse': [2898], 'fluorescence': [2899], 'microscope': [2900], 'equipped': [2901], 'charge-coupled': [2904], 'device': [2905], '(CCD)': [2906], 'camera': [2907], '(Nikon,': [2908], 'Melville,': [2909], 'NY).': [2910], 'permeabilize': [2913], 'membrane,': [2916], 'incubated': [2919, 2979, 3204], 'buffer': [2928, 3103, 3231], '(1%': [2929], 'BSA,': [2930], '0.3': [2931], 'msucrose,': [2932], 'm': [2934, 3162], 'KCl,': [2935], '2.5': [2936], 'mm': [2937, 2940, 2943, 3087, 3105, 3108, 3129, 3165, 3168, 3233, 3238, 3243], 'MgCl2,': [2938], '1': [2939, 3123, 3206, 3242, 3445, 3523], 'EDTA,': [2941, 3169, 3244], 'HEPES,': [2944], 'pH': [2945, 3110, 3235], '7.4)': [2946], '0,': [2948], '1,': [2949], '1.5,': [2950], 'digitonin': [2954, 3968], 'concentrations': [2959, 3916, 3966], 'α-Gal': [2964, 3948, 3988, 4013, 4075, 4090, 4157], 'used': [2967, 3799, 4022, 4433], 'permeabilization.': [2970], 'before': [2986], 'addition': [2987, 3550], 'visualization': [2992], 'constructs,': [3000], '10-cm': [3006], 'diameter': [3007], 'dishes': [3008], 'DNA,': [3015], '20': [3016], 'μl': [3017, 3022], 'reagent,': [3025], "manufacturer's": [3029], 'recommendations.': [3030], 'transiently': [3032, 3813], 'expressed': [3033, 3541, 3814, 3853, 3862, 4033, 4386, 4392, 4442], 'analyzed': [3036, 3310, 4036, 4118, 4388], 'immunoprecipitation': [3038], 'SDS-polyacrylamide': [3040], '(PAGE),': [3043], 'essentially': [3044], 'Briefly,': [3066], 'metabolically': [3074], 'labeled': [3075], '[35S]methionine': [3077], '16': [3079], 'h.': [3080], 'harvested': [3083], 'EDTA': [3088], 'centrifuged': [3090, 3142], '800': [3092], 'gfor': [3094], 'pellet': [3099, 3154], 'resuspended': [3101, 3156, 3249], '(150': [3104], 'NaCl,': [3106, 3163, 3239], 'Tris,': [3109, 3166, 3234], '8.0,': [3111], 'mmEDTA,': [3113], 'leupeptin,': [3116], 'pepstatin': [3119], 'A,': [3120, 3426], '25': [3121], 'μg/mlN-acetyl-leucinal-leucinal-norleucinal,': [3122], 'μm/ml': [3124], 'phenylmethylsulfonyl': [3125], 'fluoride,': [3126], 'Pefabloc),': [3130], 'disrupted': [3131], 'sonication': [3133], 'Branson': [3136], 'sonifier': [3137], '(Smith-Klein,': [3138], 'Philadelphia,': [3139], 'PA),': [3140], '22,000': [3144, 3194], 'g': [3146, 3196], 'resultant': [3151], '0.5': [3158, 3161], 'ml': [3159], 'Igepal,': [3171, 3241], 'supplemented': [3172], 'above': [3175, 4462], 'proteinase': [3176], 'inhibitors,': [3177], 'passages': [3180], 'through': [3181], '22-gauge': [3183], 'needle,': [3184], 'rocking': [3187], 'centrifugation': [3192], 'solubilized': [3201], 'membranes': [3202], '2.8': [3209], 'M2,': [3213], '45-min': [3217], 'G-agarose': [3221], 'beads': [3222], '(Roche': [3223], 'Biochemicals).': [3225], 'immunoprecipitates': [3227], '(10': [3232], '7.5,': [3236], '150': [3237], '0.1%': [3240], '0.25%': [3245], 'gelatin,': [3246], '0.2%': [3247], 'NaN3),': [3248], 'SDS-gel': [3251], 'loading': [3252], 'buffer,': [3253], 'heated': [3254], '65': [3259], '°C,': [3260], 'subjected': [3262, 3894], 'SDS-PAGE': [3264], '(28Laemmli': [3266], 'E.K.': [3267], '1970;': [3269], '227:': [3270], '680-685Crossref': [3271], '(207537)': [3274], 'N-Glycosidase': [3277], 'F': [3278], 'digestions': [3279], 'carried': [3281, 3834], 'out': [3282, 3835], 'identify': [3312], 'domains.': [3318], 'Using': [3319], 'TMpred': [3321], 'algorithm': [3322], '(29Hofmann': [3323], 'Stoffel': [3325], 'W.': [3326], '1993;': [3329], '347:': [3330], '166Google': [3331], 'EMBnet-CH,': [3334], 'evaluated.': [3347], 'A).': [3359], 'assignment': [3361], '4–8': [3366], 'based': [3368], 'relative': [3371], 'hydrophobicity': [3372], 'high': [3375], 'degree': [3376], 'similarity': [3378], 'SCAP': [3384], '(30Olender': [3387], 'E.H.': [3388], 'Simoni': [3389], '1992;': [3394], '267:': [3395], '4223-4235Abstract': [3396], '31Nohturfft': [3403], '273:': [3413], '17243-17250Abstract': [3414], '(164)': [3422], 'SSD).': [3427], 'Based': [3428], 'observations': [3431], 'computer': [3433], 'analyses,': [3434], 'Fig.': [3444, 4041, 4404], 'Band': [3446], 'consists': [3447], 'ranging': [3455], 'size': [3457], '∼50': [3459], '(loop': [3462, 3468, 3494], 'B)': [3463], '∼265': [3465], 'A),': [3469], '27-amino': [3472], 'endosomal/lysosomal': [3479, 3778], 'dileucine': [3480], 'targeting': [3481], 'motif.': [3482], 'predicts': [3487], 'remains': [3496], 'upon': [3500], 'entering': [3501], '(ER).': [3505], 'locations': [3509, 4031], 'B),': [3524], 'series': [3526], 'epitope-tagged,': [3528], 'analyzed.': [3535], 'determine': [3547, 3573, 3801, 3872], 'whether': [3548, 3574, 3861], 'tags': [3553, 4109, 4177], 'interfered': [3554], 'processing,': [3558, 3846], 'transport,': [3559], 'and/or': [3560], 'function.': [3561], 'Transfected': [3562], 'expressing': [3576], 'corrected': [3581], 'thus': [3586, 3704, 4165], 'producing': [3587], 'filipin-negative': [3588], '50–100': [3590], 'NPC1-positive': [3591], 'scored': [3594], 'correction.': [3596], '∼60–90%': [3600], 'positive': [3602, 3636], 'appeared': [3604, 4292], 'corrected,': [3607], 'exception': [3610, 3767], 'about': [3618], '22%': [3619], 'corrected.': [3623], 'Representative': [3624, 4055], 'fields': [3625], 'each': [3627, 4060], 'Fig.3;': [3632], '(left': [3640], 'panels;': [3641], 'arrows)': [3642], 'dramatic': [3645], 'decrease': [3646], 'staining': [3649, 4069, 4291], '(right': [3650], 'panels;arrows)': [3651], 'compared': [3652], 'neighboring': [3654], 'negative': [3658, 4025, 4156], 'expression.': [3661], 'Only': [3662], 'construct,': [3664, 3772], 'C),': [3674], 'did': [3675, 4394], 'complement': [3679], 'phenotype.': [3682], 'Upon': [3683], 'careful': [3684], 'inspection': [3685], 'clear': [3688], 'colocalize': [3698], 'stored': [3701], 'unable': [3706], 'mobilize': [3708], 'cholesterol,': [3709], 'similar': [3710], 'mutagenesis': [3715], '(32Watari': [3720], 'Glick': [3726], '805-810Crossref': [3748], '(127)': [3751], 'and,': [3764], 'correctly': [3774], 'targeted': [3775], 'system,': [3779], 'Thus,': [3788], 'remained': [3793], 'active': [3795], 'could': [3797], 'NPC1.': [3806], 'electroporation.': [3819], 'Because': [3820], 'easy': [3824], 'maintain,': [3828], 'majority': [3830], 'experiments': [3832], 'Initial': [3839], 'showed': [3841], 'no': [3842, 3903, 4089], 'differences': [3843], 'distribution,': [3847], 'integrity': [3850], 'topologically': [3859], 'simian': [3866], '(data': [3868, 4457], 'shown).': [3870, 3999, 4459], 'positions': [3874], 'microscopy': [3884], 'used.': [3886, 4369], 'under': [3898], 'three': [3901], 'conditions:': [3902], '(absence': [3905], 'detergent);': [3907], 'digitonin;': [3918], 'full': [3920], 'fixation.': [3924], 'fluorescein-conjugated': [3937], 'antibody.': [3939, 3961], 'As': [3940, 4401], 'control': [3942, 4015, 4026], 'intralysosomal': [3946], 'specific': [3954], 'rhodamine-conjugated': [3959], 'Pilot': [3962], 'below': [3969], 'specifically': [3972], 'but': [3977, 4131], 'membranes,': [3980], 'inability': [3985], 'detect': [3992, 4409, 4435], 'target': [3994], 'within': [3996, 4382], '(not': [3998], 'Under': [4000], 'conditions,': [4002], 'cytosolic': [4003, 4169], 'epitopes': [4005], 'while': [4008, 4027], 'ones,': [4010], 'negative.': [4018], 'Nonpermeabilized': [4019], 'efficiency': [4029], 'fully': [4038, 4063, 4130], 'shows': [4043], 'detection': [4048, 4144], 'constructs.': [4054], 'condition.': [4061], 'bright,': [4067], 'vesicular': [4068], 'intraorganelle': [4073], 'marker': [4074], 'observed,': [4078], 'access.': [4082], 'digitonin-permeabilized': [4085, 4151], 'there': [4087], 'staining,': [4092], 'confirming': [4093], 'membrane.': [4101, 4227, 4400], 'six': [4103], 'A–E': [4112], 'tail,': [4116], '4).': [4120], 'Detection': [4121], 'A-tagged': [4125], 'partially': [4134], 'luminal.': [4142, 4424], 'Similarly,': [4143], 'B-tagged': [4148], 'signal,': [4159], 'epitope,': [4163], 'B,': [4167], 'contrast,': [4174], 'D': [4182, 4206, 4234], 'undetectable': [4185], 'digitonin-treated': [4187, 4414], 'location': [4192, 4361, 4453], '4,': [4194], 'D).': [4197], 'reversed': [4210, 4322], 'respect': [4212], 'model,': [4216], 'implying': [4217], 'TM3': [4221, 4468], 'span': [4225], 'suggests': [4229], 'comprise': [4235], 'shifted': [4244], 'position': [4248, 4381], 'one.': [4252], 'location,': [4261], 'assuming': [4262], 'elimination': [4263, 4466], 'TM3.': [4265], 'distribution': [4268], 'surprising.': [4270], 'quantities': [4277], 'nonpermeabilized': [4283], 'E).': [4287, 4333], 'speckled': [4293], 'uneven,': [4295], 'perhaps': [4296], 'some': [4299], 'form': [4300], 'aggregation': [4302], 'occurred': [4303], 'surface.': [4307], 'phenomenon': [4310], 'apparently': [4313], 'interfere': [4314], 'function,': [4317], 'because': [4318], 'successfully': [4321], 'storage': [4325], 'observed': [4326], 'filipin-stained': [4328], 'mechanism': [4335], 'causes': [4345], 'mislocalization': [4346], 'being': [4355], 'investigated.': [4356], 'confirm': [4359], 'different': [4366, 4380], 'approaches': [4367], 'First,': [4370], 'mislocalize': [4396], 'failure': [4407], 'previous': [4418], 'conclusion': [4419], 'Second,': [4425], 'directed': [4428], 'against': [4429], 'lending': [4447], 'Notably,': [4460], 'reverses': [4473], 'SSD.': [4478], 'orientation,': [4481], 'i': [4488]}, 'cited_by_api_url': 'https://api.openalex.org/works?filter=cites:W1967383774', 'counts_by_year': [{'year': 2024, 'cited_by_count': 7}, {'year': 2023, 'cited_by_count': 8}, {'year': 2022, 'cited_by_count': 9}, {'year': 2021, 'cited_by_count': 6}, {'year': 2020, 'cited_by_count': 14}, {'year': 2019, 'cited_by_count': 15}, {'year': 2018, 'cited_by_count': 10}, {'year': 2017, 'cited_by_count': 19}, {'year': 2016, 'cited_by_count': 17}, {'year': 2015, 'cited_by_count': 10}, {'year': 2014, 'cited_by_count': 6}, {'year': 2013, 'cited_by_count': 14}, {'year': 2012, 'cited_by_count': 18}], 'updated_date': '2024-12-15T23:20:56.293694', 'created_date': '2016-06-24'}