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1486, 1515, 1548, 1584, 1610, 1644, 1686, 1722, 1766, 1809, 2175, 2543, 2862, 3075, 3726, 3923], 'this': [62, 114, 132, 152, 174, 202, 229, 272, 298, 344, 362, 384, 409, 441, 467, 499, 533, 591, 620, 653, 689, 715, 749, 791, 827, 871, 914, 958, 1010, 1028, 1048, 1070, 1098, 1125, 1168, 1194, 1240, 1258, 1280, 1305, 1337, 1363, 1395, 1429, 1487, 1516, 1549, 1585, 1611, 1645, 1687, 1723, 1767, 1810, 2105, 2232, 2342, 2608, 2788, 2923, 3678, 3979], 'author': [63, 115, 133, 153, 175, 203, 230, 273, 299, 345, 363, 385, 410, 442, 468, 500, 534, 592, 621, 654, 690, 716, 750, 792, 828, 872, 915, 959, 1011, 1029, 1049, 1071, 1099, 1126, 1169, 1195, 1241, 1259, 1281, 1306, 1338, 1364, 1396, 1430, 1488, 1517, 1550, 1586, 1612, 1646, 1688, 1724, 1768, 1811, 2150], 'Frank': [64, 66, 960, 962, 1816], 'Bienaimé': [65, 67, 961, 963], 'Service': [101, 997], "d'Explorations": [102, 998, 1909], 'Fonctionnelles,': [103, 999, 1910], 'Hôpital': [104, 1000, 1911], 'Necker-Enfants': [105, 480, 1001, 1376, 1912, 2004], 'Kamile': [116, 118, 1012, 1014, 1818], 'Lukas': [117, 119, 1013, 1015], 'Abhijeet': [134, 137, 1030, 1033, 1820], 'P': [135, 138, 1031, 1034, 1821], 'Todkar': [136, 139, 1032, 1035], 'Manuel': [154, 156, 1050, 1052, 1823], 'Knoll': [155, 157, 1051, 1053], 'Rheumatology': [160, 370, 843, 854, 1056, 1266, 1739, 1750, 1918, 2088], 'Clinical': [162, 372, 845, 856, 1058, 1268, 1741, 1752, 1920, 2090], 'Immunology,': [163, 373, 846, 857, 1059, 1269, 1742, 1753, 1921, 2091], 'Toma': [176, 179, 1072, 1075, 1825], 'A': [177, 180, 1073, 1076, 1826, 2605], 'Yakulov': [178, 181, 1074, 1077], 'Alexis': [204, 206, 1100, 1102, 1828], 'Hofherr': [205, 207, 1101, 1103], 'orcid.org/0000-0003-2842-6161': [208, 1104], 'Oliver': [231, 233, 1127, 1129, 1830], 'Kretz': [232, 234, 1128, 1130], 'Neuroanatomy,': [252, 1148, 1929], 'Albert-Ludwigs-University': [253, 458, 514, 524, 572, 582, 636, 818, 905, 1149, 1354, 1410, 1420, 1468, 1478, 1532, 1714, 1801, 1930, 1993, 2027, 2037, 2047], 'III.': [257, 556, 674, 1153, 1452, 1570], 'Center': [263, 335, 449, 505, 518, 562, 566, 576, 627, 680, 731, 764, 812, 899, 1159, 1231, 1345, 1401, 1414, 1458, 1462, 1472, 1523, 1576, 1627, 1660, 1708, 1795, 1940, 1967], 'Hamburg-Eppendorf,': [264, 563, 681, 1160, 1459, 1577, 1941], 'Hamburg,': [265, 564, 682, 1161, 1460, 1578, 1942], 'Martin': [274, 276, 1170, 1172, 1832], 'Helmstädter': [275, 277, 1171, 1173], 'Wilfried': [300, 302, 1196, 1198, 1834], 'Reichardt': [301, 303, 1197, 1199], 'Physics,': [313, 391, 1209, 1287, 1945], 'Radiology,': [316, 394, 1212, 1290, 1948], 'Comprehensive': [318, 396, 1214, 1292, 1950], 'Cancer': [319, 327, 333, 397, 723, 729, 756, 762, 1215, 1223, 1229, 1293, 1619, 1625, 1652, 1658, 1951, 1959, 1965], 'German': [326, 332, 722, 728, 755, 761, 1222, 1228, 1618, 1624, 1651, 1657], 'Consortium': [328, 724, 757, 1224, 1620, 1653, 1960], '(DKTK),': [329, 725, 758, 1225, 1621, 1654, 1961], 'Research': [334, 730, 763, 781, 1230, 1626, 1659, 1677, 1966, 2081], '(DKFZ),': [336, 732, 765, 1232, 1628, 1661, 1968], 'Heidelberg,': [337, 733, 766, 1233, 1629, 1662, 1969], 'Simone': [346, 348, 1242, 1244, 1836], 'Braeg': [347, 349, 1243, 1245], 'Tom': [364, 366, 1260, 1262, 1838], 'Aschman': [365, 367, 1261, 1263], 'Annette': [386, 388, 1282, 1284, 1840], 'Merkle': [387, 389, 1283, 1285], 'Dietmar': [411, 413, 1307, 1309, 1842], 'Pfeifer': [412, 414, 1308, 1310], 'Hematology,': [425, 1321, 1973], 'Oncology': [426, 1322, 1974], 'Stem': [428, 1324, 1976], 'Cell': [429, 780, 1325, 1676, 1977, 2080], 'Transplantation,': [430, 1326, 1978], 'Verónica': [443, 446, 1339, 1342, 1844], 'I': [444, 447, 1340, 1343, 1845], 'Dumit': [445, 448, 1341, 1344], 'Biological': [451, 507, 520, 568, 578, 629, 814, 901, 1347, 1403, 1416, 1464, 1474, 1525, 1710, 1797, 1986, 2020, 2033, 2043], 'Systems': [452, 508, 579, 630, 768, 1348, 1404, 1475, 1526, 1664, 1987, 2021, 2044], 'Analysis': [453, 509, 580, 631, 1349, 1405, 1476, 1527, 1988, 2022, 2045], '(ZBSA),': [454, 510, 581, 632, 1350, 1406, 1477, 1528, 1989, 2023, 2046], 'Core': [455, 633, 1351, 1529, 1990], 'Facility': [456, 634, 1352, 1530, 1991], 'Proteomics,': [457, 635, 1353, 1531, 1992], 'Marie-Claire': [469, 471, 1365, 1367, 1847], 'Gubler': [470, 472, 1366, 1368], 'UMR1163,': [474, 1370, 1998], 'Laboratory': [475, 1371, 1999], 'Inherited': [477, 1373, 2001], 'Kidney': [478, 1374, 2002, 2398, 2553, 3905], 'Diseases,': [479, 1375, 2003], 'Malades': [481, 1377, 2005], 'Hospital,': [482, 1378, 2006], 'Imagine': [485, 1381], 'Institute,': [486, 1382, 2010], 'Roland': [501, 503, 1397, 1399, 1849], 'Nitschke': [502, 504, 1398, 1400], 'Life': [511, 1407, 2024], 'Imaging': [512, 1408, 2025], 'Signaling': [521, 569, 815, 902, 1417, 1465, 1711, 1798, 2034], 'Studies': [522, 570, 816, 903, 1418, 1466, 1712, 1799, 2035], '(BIOSS),': [523, 571, 817, 904, 1419, 1467, 1713, 1800, 2036], 'Tobias': [535, 538, 1431, 1434, 1851], 'B': [536, 539, 1432, 1435, 1852], 'Huber': [537, 540, 1433, 1436], 'Fabiola': [593, 595, 1489, 1491, 1854], 'Terzi': [594, 596, 1490, 1492], 'Jörn': [622, 624, 1518, 1520, 1856], 'Dengjel': [623, 625, 1519, 1521], 'orcid.org/0000-0002-9453-4614': [626, 1522], 'Biology,': [642, 1538, 2053], 'Fribourg,': [645, 646, 1541, 1542, 2056, 2057], 'Switzerland': [647, 1543, 2058], 'Florian': [655, 657, 1551, 1553, 1858], 'Grahammer': [656, 658, 1552, 1554], 'Michael': [691, 693, 1587, 1589, 1860], 'Köttgen': [692, 694, 1588, 1590], 'Hauke': [717, 719, 1613, 1615, 1862], 'Busch': [718, 720, 1614, 1616], 'orcid.org/0000-0003-4763-4521': [721, 1617], 'Institute': [735, 775, 1631, 1671, 2075], 'Experimental': [737, 1633, 2061], 'Dermatology,': [738, 1634, 2062], 'Lübeck,': [741, 742, 1637, 1638, 2065, 2066], 'Melanie': [751, 753, 1647, 1649, 1864], 'Boerries': [752, 754, 1648, 1650], 'Biology': [769, 1665, 2069], 'Cellular': [772, 1668, 2072], 'Microenvironment': [773, 1669, 2073], 'Group,': [774, 1670, 2074], 'Molecular': [777, 1673, 2077], 'Medicine': [778, 1674, 2078], '(IMMZ),': [782, 1678, 2082], 'Albert-Ludwigs-University,': [783, 1679, 2083], 'Gerd': [793, 795, 1689, 1691, 1866], 'Walz': [794, 796, 1690, 1692, 2990], 'Antigoni': [829, 831, 1725, 1727, 1868], 'Triantafyllopoulou': [830, 832, 1726, 1728], 'Charité': [858, 1754, 2092], '-': [859, 1755, 2093], 'Centre': [862, 1758, 2096], 'Berlin,': [863, 864, 1759, 1760, 2097, 2098], 'E': [873, 878, 1769, 1774, 1871], 'Wolfgang': [874, 879, 1770, 1775, 1872], 'Kuehn': [875, 880, 1771, 1776, 1873], 'Corresponding': [876, 1772], 'Author': [877, 1773, 1812], '[email': [881, 1777, 2120], 'protected]': [882, 1778, 2121], 'orcid.org/0000-0002-7753-9616': [883, 1779], 'Information': [1813], 'Viau1,2,3,‡,': [1815], 'Bienaimé1,2,3,4,‡,': [1817], 'Lukas1,': [1819], 'Todkar1,': [1822], 'Knoll5,': [1824], 'Yakulov1,2,': [1827], 'Hofherr1,2,': [1829], 'Kretz1,2,6,7,': [1831], 'Helmstädter1,2,': [1833], 'Reichardt2,8,9,10,': [1835], 'Braeg1,': [1837], 'Aschman5,': [1839], 'Merkle8,': [1841], 'Pfeifer2,11,': [1843], 'Dumit12,': [1846], 'Gubler13,14,': [1848], 'Nitschke15,16,': [1850], 'Huber1,2,7,16,17,': [1853], 'Terzi3,': [1855], 'Dengjel12,18,': [1857], 'Grahammer1,2,7,': [1859], 'Köttgen1,2,': [1861], 'Busch9,10,19,': [1863], 'Boerries9,10,20,': [1865], 'Walz1,2,16,': [1867], 'Triantafyllopoulou2,5,21': [1869], '*,1,2,16': [1874], '1Renal': [1875], '2Faculty': [1882], '3INSERM': [1890], '4Service': [1908], '5Department': [1916], '6Department': [1927], '7III.': [1934], '8Medical': [1944], '9German': [1958], '10German': [1964], '11Department': [1971], '12Center': [1984], '13INSERM': [1997], '14Imagine': [2009], '15Center': [2018], '16Center': [2031], '17Center': [2041], '18Department': [2051], '19Institute': [2059], '20Systems': [2068], '21Department': [2086], '‡These': [2100], 'authors': [2101], 'contributed': [2102], 'equally': [2103], 'to': [2104, 2152, 2155, 2189, 2209, 2311, 2421, 2445, 2451, 2490, 2596, 2607, 2853, 2886, 2888, 2921, 2964, 2979, 2984, 3032, 3042, 3101, 3124, 3257, 3301, 3352, 3676, 4026], 'work': [2106], '*Corresponding': [2107], 'author.': [2108], 'Tel:': [2109], '+49': [2110, 2115], '761': [2111, 2116], '270': [2112, 2117], '32270;': [2113], 'Fax:': [2114], '32860;': [2118], 'E-mail:': [2119], 'The': [2122, 2316, 2351, 2396, 2561, 2874, 3159, 3219, 3711, 4037], 'EMBO': [2123], 'Journal': [2124], '(2018)37:e98615https://doi.org/10.15252/embj.201798615': [2125], 'PDFDownload': [2126], 'PDF': [2127], 'article': [2129], 'text': [2130], 'main': [2132], 'figures.': [2133], 'Peer': [2134], 'ReviewDownload': [2135], 'a': [2136, 2186, 2215, 2258, 2271, 2283, 2301, 2326, 2355, 2422, 2673, 2680, 2723, 2863, 2936, 3025, 3107, 3120, 3163, 3199, 3260, 3282, 3357, 3991, 4016], 'summary': [2137], 'editorial': [2140, 2144], 'decision': [2141, 2145], 'including': [2143, 2226, 2666], 'letters,': [2146], 'reviewer': [2147], 'comments': [2148], 'responses': [2151], 'feedback.': [2153], 'ToolsAdd': [2154], 'favoritesDownload': [2156], 'CitationsTrack': [2157], 'CitationsPermissions': [2158], 'ShareFacebookTwitterLinked': [2159], 'InMendeleyWechatReddit': [2160], 'Figures': [2161], '&': [2162, 2461, 2582, 2688, 2692, 2706, 2829], 'Info': [2163], 'Abstract': [2164], 'Polycystic': [2165, 2397, 2417, 2552], 'disease': [2167, 2294, 2347, 2454, 2473, 2598], '(PKD)': [2168, 2400], 'other': [2170], 'renal': [2171, 2204, 2252, 2297, 2418, 2667, 2784, 3020, 3026, 3055, 3164, 3194, 3265, 3293, 3310, 3353, 3358, 3371, 4023], 'ciliopathies': [2172, 2298, 2626], 'are': [2173, 2511, 2655, 2812, 2860, 2916, 3255, 3723, 3975], 'characterized': [2174, 2861, 3074], 'cysts,': [2176, 2601, 2798, 3043, 3344], 'inflammation,': [2177, 2602, 3044, 3246], 'fibrosis.': [2179, 2604, 3046], 'Cilia': [2180, 2671], 'function': [2181, 3058, 3311, 3328], 'as': [2182, 2282, 2468, 2697, 2731, 3083, 3085], 'signaling': [2183, 2208, 2882, 2982], 'centers,': [2184], 'but': [2185, 3148, 3785], 'molecular': [2187, 3034], 'link': [2188], 'inflammation': [2190, 2808, 2915, 3347, 3375, 3588], 'has': [2194, 2529, 2566, 2883], 'not': [2195, 3431, 3724, 3786, 3968], 'been': [2196, 2530, 2884], 'established.': [2197], 'Here,': [2198], 'we': [2199, 3010, 3017, 3030, 4014], 'show': [2200, 3319], 'that': [2201, 2335, 2357, 2437, 2615, 2880, 2956, 3185, 3272, 3320, 3722, 4032], 'cilia': [2202, 2412, 2643, 2654, 2779, 2941, 2959, 2977, 3136, 3154, 3436, 3766, 3788, 3812], 'epithelia': [2205], 'activate': [2206], 'recruit': [2210], 'inflammatory': [2211, 2804], 'cells.': [2212, 2244], 'We': [2213, 2904, 2950, 3104], 'identify': [2214, 4027], 'complex': [2216, 2327, 2370, 2681], 'ciliary': [2219, 2233], 'kinase': [2220, 2333, 2927, 2929, 2933], 'several': [2223], 'ciliopathy-related': [2224, 2329], 'proteins': [2225, 2330, 2617, 3041], 'NPHP1': [2227, 3998], 'PKD1.': [2229], 'At': [2230], 'homeostasis,': [2231], 'module': [2234], 'suppresses': [2235], 'expression': [2236, 2256, 2359, 2387, 3698], 'CCL2': [2240, 2255, 2386], 'tubular': [2242, 2414, 3242, 3342, 3581], 'epithelial': [2243, 2278, 2306], 'Deletion': [2245, 3048], 'or': [2248, 2383, 2413, 2548], 'PKD1': [2249, 2404, 2547], 'mouse': [2251, 3270, 3368], 'tubules': [2253, 2668, 3577, 3770, 3781, 3791], 'elevates': [2254], 'cell-autonomous': [2259], 'manner': [2260], 'results': [2262, 3338], 'peritubular': [2264, 2389], 'accumulation': [2265], 'CCR2+': [2267], 'mononuclear': [2268], 'phagocytes,': [2269], 'promoting': [2270], 'ciliopathy': [2272, 2394, 3027, 3359, 3988], 'phenotype.': [2273, 3028, 3360], 'Our': [2274], 'findings': [2275, 3254, 3318], 'establish': [2276], 'an': [2277, 2292, 2345, 2368, 2676, 2803, 3070], 'organelle,': [2279], 'cilium,': [2281], 'gatekeeper': [2284], 'immune': [2287, 2309, 2319], 'cell': [2288, 2320, 2613, 2664, 2732, 2735], 'numbers.': [2289], 'This': [2290, 2363], 'represents': [2291, 3654, 3950], 'unexpected': [2293, 2346], 'mechanism': [2295, 2348], 'establishes': [2300], 'new': [2302], 'model': [2303], 'how': [2305, 2775, 2909], 'cells': [2307, 2310], 'regulate': [2308, 2337, 2980], 'affect': [2312, 2628, 2722], 'homeostasis.': [2314], 'Synopsis': [2315], 'cilium': [2317, 2353], 'instructs': [2318], 'behaviour': [2321], 'via': [2325], 'together': [2336], 'signalling.': [2339], 'Dysregulation': [2340], 'system': [2343, 2684], 'presents': [2344], 'ciliopathies.': [2350, 2428, 2621, 2785], 'primary': [2352, 2653, 3435, 3811], 'transmits': [2354], 'signal': [2356, 2364, 2698, 2912], 'activates': [2358, 2962], 'CCL2.': [2362, 2415], 'is': [2365, 2406, 2506, 2541, 2570, 2773, 2925, 2935, 3322, 3430, 3763, 3797], 'regulated': [2366], 'through': [2367], 'intra-ciliary': [2369], 'LKB1,': [2372, 3037], 'NPHP1,': [2373], 'polycystin': [2374], '1': [2375, 3563, 3866], '(PC1),': [2376], 'ANKS3': [2377], 'NEK7.': [2379], 'Loss': [2380, 3332, 3363], 'PC1': [2384], 'increases': [2385], 'numbers': [2391, 2486], 'promotes': [2393, 2845], 'phenotypes.': [2395], 'Disase': [2399], 'phenotype': [2401, 2782, 4007], 'seen': [2402], 'upon': [2403], 'depletion': [2405], 'ameliorated': [2407], 'absence': [2410], 'Introduction': [2416], 'diseases': [2419], 'belong': [2420], 'spectrum': [2423], 'inherited': [2425, 3071], 'disorders': [2426, 2436, 2481], 'termed': [2427], 'They': [2429, 2695], 'include': [2430], 'genetically': [2431], 'distinct': [2432], 'morphologically': [2434], 'heterogeneous': [2435], 'manifest': [2438], 'at': [2439, 2659, 2973, 3172, 3225, 3387, 3454, 3470, 3483, 3500, 3532, 3708, 3823, 3851, 3884, 3898, 3911, 3925], 'any': [2440], 'time': [2441, 3211], 'from': [2442, 2623, 2797, 3134, 3382, 3417, 3525, 3614, 3685, 3703, 3716, 3750, 3768, 3789, 3871, 3893, 4022, 4041], 'early': [2443, 2842], 'childhood': [2444], 'late': [2446, 2856], 'life': [2448], 'lead': [2450], 'end-stage': [2452], 'majority': [2457], 'cases': [2459], '(Braun': [2460], 'Hildebrandt,': [2462, 2583, 2830], '2016).': [2463, 2584, 2770, 2835, 2903, 3001], 'In': [2464, 2500, 2806, 3640, 3658, 3932], 'adults,': [2465], 'they': [2466], 'present': [2467, 3191, 3764], 'autosomal': [2469], 'dominant': [2470], 'polycystic': [2471], '(ADPKD),': [2474], 'one': [2475, 3655, 3951], 'most': [2478, 2503, 2663], 'common': [2479], 'monogenetic': [2480], 'man,': [2483], 'with': [2484, 3119, 3303, 3348, 3987], 'large': [2485], 'cysts': [2488, 2520, 2817, 3201, 3222], 'leading': [2489, 3100, 3351], 'massive': [2491], 'enlargement': [2492, 2847], 'kidneys': [2495, 2510, 3206, 3381, 3684, 3892], '(Ong': [2496], 'et': [2497, 2524, 2558, 2574, 2578, 2639, 2710, 2714, 2718, 2740, 2744, 2748, 2752, 2756, 2760, 2764, 2768, 2819, 2823, 2833, 2849, 2871, 2893, 2897, 2901, 2943, 2947, 2987, 2991, 2995, 2999, 3091, 3116, 3277], 'al,': [2498, 2525, 2559, 2575, 2579, 2640, 2711, 2715, 2719, 2741, 2745, 2749, 2753, 2757, 2761, 2765, 2769, 2820, 2824, 2834, 2850, 2872, 2894, 2898, 2902, 2944, 2948, 2988, 2992, 2996, 3000, 3092, 3117, 3278], '2015).': [2499, 2873, 2949], 'children,': [2501], 'prevalent': [2504], 'form': [2505], 'nephronophthisis': [2507], '(NPHP).': [2508], 'These': [2509, 3253, 3317], 'mostly': [2512], 'small': [2513], 'fibrotic': [2515], 'contain': [2517, 2616, 2672], 'only': [2518], 'few': [2519, 2587, 3200], '(Wolf,': [2521], '2015;': [2522, 2827], 'Konig': [2523], '2017).': [2526, 2694], 'Important': [2527], 'progress': [2528], 'made': [2531], 'identifying': [2533], 'genetic': [2535], 'causes': [2536, 3374], 'these': [2538, 2593, 3138, 4042], 'entities:': [2539], 'ADPKD': [2540, 2799, 2838, 2859, 2889], 'caused': [2542], 'mutations': [2544, 3060], 'either': [2546], 'PKD2': [2549], '(The': [2550], 'International': [2551], 'Disease': [2554], 'Consortium,': [2555], '1995;': [2556], 'Mochizuki': [2557], '1996).': [2560], 'number': [2562, 3150], 'NPHP': [2564, 2801, 2891], 'genes': [2565, 2595], 'surpassed': [2567], '20': [2568, 3861], 'growing': [2571], 'annually': [2572], '(Hildebrandt': [2573, 2638, 2818], '1997;': [2576, 2580, 2821, 2825], 'Saunier': [2577, 2822], 'Braun': [2581, 2828], 'Yet,': [2585], 'very': [2586], 'insights': [2588, 4001], 'exist': [2589], 'into': [2590, 4002], 'what': [2591, 4003], 'links': [2592], 'diverse': [2594], 'manifestations': [2599], 'key': [2606], 'question': [2609, 2908, 2924], 'lies': [2610], 'cilia,': [2612, 2910, 3038], 'organelles': [2614], 'mutated': [2618], 'Apart': [2622], 'disease,': [2625, 3069], 'also': [2627, 3402, 3446, 3491, 3605], 'liver,': [2630], 'central': [2632], 'nervous': [2633], 'system,': [2634], 'skeleton': [2637], '2011).': [2641, 2851], 'Motile': [2642], 'occur': [2644], 'bundles': [2646], 'airways': [2649], 'brain,': [2651], 'whereas': [2652], 'non-motile': [2656], 'filiform': [2657], 'structures': [2658], 'surface': [2661], 'types,': [2665], '(Drummond,': [2669], '2012).': [2670], 'microtubule-based': [2674], 'cytoskeleton,': [2675], 'ultrastructural': [2677], 'gate,': [2678], 'macromolecular': [2682], 'transport': [2683], '(Nachury,': [2685], '2014;': [2686, 2997], 'Stepanek': [2687], 'Pigino,': [2689], '2016;': [2690, 2831], 'Garcia-Gonzalo': [2691], 'Reiter,': [2693], 'act': [2696], 'transducers': [2699], 'receiving': [2700], 'physical': [2701], 'chemical': [2703], 'stimuli': [2704], '(Praetorius': [2705], 'Spring,': [2707], '2001;': [2708], 'Corbit': [2709], '2005;': [2712], 'Lechtreck': [2713], '2009;': [2716, 2758], 'Boehlke': [2717, 2759], '2010b)': [2720], 'wide': [2724], 'range': [2725], 'autonomous': [2727], 'cellular': [2728], 'functions': [2729], 'such': [2730], 'size,': [2733], 'polarity,': [2734], 'cycle,': [2736], 'migration': [2738], '(Kim': [2739], '2004;': [2742], 'Park': [2743], '2006;': [2746], 'Robert': [2747], '2007;': [2750], 'Jones': [2751], '2008;': [2754], 'Vasilyev': [2755], '2010b;': [2762, 2945, 2989], 'Schneider': [2763], '2010;': [2766, 2993], 'Orhon': [2767, 2998], 'However,': [2771], 'it': [2772], 'unclear': [2774], 'dysfunction': [2777], 'explains': [2780], 'When': [2786], 'exploring': [2787], 'question,': [2789], 'two': [2790], 'observations': [2791], 'prompted': [2792], 'our': [2793], 'attention.': [2794], 'First,': [2795, 3016], 'apart': [2796], 'share': [2802], 'phenotype:': [2805], 'NPHP,': [2807, 2854], 'subsequent': [2810], 'fibrosis': [2811, 2867, 3248, 3377, 3593], 'much': [2813], 'prominent': [2815], 'than': [2816], 'Wolf,': [2826], 'Slaats': [2832], 'Likewise,': [2836], 'infiltration': [2840], 'occurs': [2841], 'on': [2843], 'cyst': [2846], '(Karihaloo': [2848], 'Similarly': [2852], 'stages': [2857], 'severe': [2864, 3097], 'degree': [2865], '(Norman,': [2868], '2011;': [2869], 'Ong': [2870], 'second': [2875], 'aspect': [2876], 'concerns': [2877], 'fact': [2879], 'metabolic': [2881, 2911, 2937, 2981], 'observed': [2885], 'contribute': [2887], '(Chaki': [2892], '2012;': [2895], 'Rowe': [2896], '2013;': [2899], 'Menezes': [2900], 'therefore': [2905, 3105], 'asked': [2906, 3018], 'transducers,': [2913], 'linked.': [2917], 'An': [2918], 'interesting': [2919], 'candidate': [2920], 'explore': [2922, 3003], 'liver': [2928], 'B1': [2930, 2934], '(LKB1).': [2931], 'Liver': [2932], 'sensor': [2938], 'localized': [2939], '(Boehlke': [2942, 2986], 'Mick': [2946], 'have': [2951], 'previously': [2952], 'shown': [2953], 'vitro': [2955], 'bending': [2957], 'under': [2960], 'flow': [2961, 3877], 'phosphorylate': [2965], 'its': [2966, 4035], 'target': [2967], 'AMPK': [2968], '(5′-adenosine': [2969], 'monophosphate-activated': [2970], 'protein': [2971, 3714, 4020], 'kinase)': [2972], 'base': [2975], 'linked': [2983], 'PKD': [2985, 3040], 'Liu': [2994], 'To': [3002, 3999], 'role': [3005], 'vivo,': [3009], 'inactivated': [3011], 'kidney.': [3015, 3331], 'whether': [3019], 'deletion': [3021, 4010], 'induces': [3024], 'Second,': [3029], 'sought': [3031], 'uncover': [3033], 'mechanisms': [3035], 'connecting': [3036], 'Results': [3047], 'disrupts': [3054, 3370], 'morphology': [3056, 3152, 3326], 'Mono-allelic': [3059], 'gene': [3063], 'STK11/LKB1': [3064], 'humans': [3066, 3302], 'cause': [3067], 'Peutz-Jeghers': [3068, 3304], 'cancer': [3072], 'syndrome': [3073], 'benign': [3076], 'tumors': [3077], 'skin': [3080], 'intestine,': [3082], 'well': [3084], 'development': [3087], 'malignancies': [3089], '(Hemminki': [3090], '1998).': [3093], 'Lkb1−/−': [3094], 'mice': [3095, 3114, 3386, 3531, 3687, 3707, 3897], 'display': [3096], 'developmental': [3098], 'defects': [3099], 'embryonic': [3102], 'lethality.': [3103], 'pursued': [3106], 'kidney-specific': [3108, 3121], 'knockout': [3109], 'strategy': [3110], 'crossed': [3112], 'Lkb1flox/flox': [3113], '(Nakada': [3115], '2010)': [3118], 'deleter': [3122], 'strain': [3123], 'excise': [3125], 'Lkb1': [3126, 3365], 'distal': [3129, 3612], 'nephron.': [3130], 'was': [3132, 3166, 3223, 3273, 3288], 'absent': [3133], 'tubule': [3139, 3613, 3718], 'segments': [3140, 3719], '(Lkb1ΔTub;': [3141], 'Figs': [3142], '1A': [3143], 'B,': [3145], 'EV1A–C),': [3147], 'were': [3155, 3190], 'unchanged': [3156], '(Fig': [3157, 3202, 3212, 3229, 3249, 3290, 3296], 'EV1D–F).': [3158], 'first': [3160], 'sign': [3161], 'disturbance': [3165], 'impaired': [3167], 'urine': [3168, 3189, 3870, 3881], 'concentration,': [3169], 'which': [3170, 3263], 'occurred': [3171], '5': [3173, 3388, 3395, 3476, 3709, 3824, 3829, 3852, 3857, 3926], 'weeks': [3174, 3472, 3505], '(Figs': [3175], '1C': [3176, 3230], 'EV1G–I);': [3178], 'magnetic': [3179], 'resonance': [3180, 3450], 'imaging': [3181], '(MRI)': [3182], 'studies': [3183], 'confirmed': [3184], 'increased': [3186, 3289], 'amounts': [3187], 'pelvis': [3195], 'occasionally': [3197], 'demonstrated': [3198], '1C).': [3203], 'Macroscopically,': [3204], 'diminished': [3207], 'size': [3209], 'over': [3210], 'EV1J': [3213], 'K;': [3215], 'Appendix': [3216, 3492], 'Fig': [3217, 3315, 3403, 3447, 3493, 3606], 'S1).': [3218, 3316], 'occurrence': [3220], 'noted': [3224], 'cortico-medullary': [3227], 'junction': [3228], 'F;': [3232], '14': [3233], '23': [3235, 3504, 3536, 3888, 3899, 3915, 3928], 'weeks).': [3236, 3560], 'Histology': [3237], 'revealed': [3238], 'dilated': [3239, 3576], 'tubules,': [3240], 'thickened': [3241, 3580], 'basement': [3243, 3582, 3623], 'membranes,': [3244], 'interstitial': [3245, 3587, 3592], '1G': [3250], 'H).': [3252], 'similar': [3256], 'those': [3258], 'previous': [3261], 'study,': [3262], 'investigated': [3264], 'metabolism': [3266], 'using': [3267], 'same': [3269], 'genotype': [3271], 'created': [3274], 'independently': [3275], '(Han': [3276], '2016).1': [3279], 'Collagen': [3280], 'synthesis,': [3281], 'marker': [3283], 'extracellular': [3285], 'matrix': [3286], 'deposition,': [3287], 'EV1L),': [3291], 'failure': [3294], 'ensued': [3295], '1D': [3297], 'E).': [3299], 'Analogous': [3300], 'syndrome,': [3305], 'heterozygote': [3306], 'animals': [3307], 'had': [3308], 'normal': [3309, 3325], 'histology': [3313], '(Appendix': [3314], 'required': [3323], 'alterations': [3340], 'architecture,': [3343], 'chronic': [3346], 'progressive': [3349], 'scarring': [3350], 'failure,': [3354], 'reminiscent': [3355], 'Figure': [3361, 3680], '1.': [3362], 'architecture': [3372], 'immunostaining': [3379], 'control': [3383, 3419, 3511, 3526, 3645, 3704, 3752, 3773, 3783, 3894, 3941], 'Lkb1ΔTub': [3385, 3424, 3439, 3516, 3530, 3541, 3598, 3616, 3650, 3686, 3706, 3757, 3777, 3793, 3896, 3946], 'weeks.': [3389, 3458, 3485, 3537, 3710, 3825, 3853, 3889, 3900, 3916, 3929, 3972], 'Representative': [3390, 3407, 3459, 3627, 3729, 3826, 3854, 3891], 'images': [3391, 3410, 3452, 3460, 3628, 3732, 3827, 3855], 'n': [3393, 3466, 3474, 3479, 3545, 3550, 3556, 3630], '=': [3394, 3421, 3426, 3467, 3475, 3480, 3513, 3518, 3546, 3551, 3557, 3631, 3754, 3759], 'mice/group.': [3396, 3830, 3858], 'Scale': [3397, 3441, 3486, 3561, 3600, 3634, 3799, 3841, 3859], 'bars:': [3398, 3442, 3487, 3635, 3800], '200': [3399], 'μm.': [3400, 3444, 3603, 3637, 3802, 3844, 3867], 'See': [3401, 3445, 3490, 3604], 'EV1A': [3404], 'B.': [3406, 3689], 'confocal': [3408, 3730], 'microscopy': [3409, 3610, 3731], 'collecting': [3412, 3815], 'duct': [3413, 3816], '(CD,': [3414], 'AQP2': [3415], 'expressing)': [3416, 3740], '5-week-old': [3418, 3751, 3872], '(n': [3420, 3425, 3512, 3517, 3753, 3758], '4)': [3422, 3755], '5)': [3427, 3760], 'animals.': [3428, 3761, 3873], 'expressed': [3432], 'CD': [3434, 3850], '(Ac-Tub)': [3437, 3767, 3813], 'mice.': [3440, 3520, 3599, 3617, 3633, 3651, 3947], '2': [3443, 3488, 3636, 3801], 'EV1C.': [3448], 'Magnetic': [3449], 'tomography': [3451], 'obtained': [3453, 4040], '10': [3455, 3471, 3558], '16': [3457, 3484], 'coronal': [3462], 'sections': [3464, 3574], '3': [3468, 3632], 'mice/group': [3469], '(control)': [3477], '8': [3481], '(Lkb1ΔTub)': [3482], 'mm.': [3489, 3564], 'S1A.': [3494], 'Plasma': [3495], 'blood': [3496], 'urea': [3497], 'nitrogen': [3498], '(BUN)': [3499], '5,': [3501, 3533, 3885, 3912], '14,': [3502, 3534, 3886, 3913], '(w).': [3506], 'Kaplan–Meier': [3507], 'survival': [3508], 'curves': [3509], '13)': [3514], '15)': [3519], 'Periodic': [3521], 'acid–Schiff': [3522], '(PAS)': [3523], 'staining': [3524], '(5': [3527, 3548], 'weeks)': [3528], 'Arrowheads': [3538], 'indicate': [3539, 3620, 3661, 3955], 'cysts.': [3540], 'mice:': [3542], 'representative': [3543], '13': [3547], 'weeks),': [3549, 3554], '12': [3552], '(14': [3553], '(23': [3559], 'bar:': [3562, 3601, 3842, 3860], 'Higher': [3565], 'magnification': [3566, 3864], 'PAS-': [3568], 'Picrosirius': [3570], 'Red': [3571], '(lower': [3572, 3589, 3594, 3625], 'right)-stained': [3573], 'showing': [3575], '(upper': [3578, 3771], 'left),': [3579, 3590], 'membranes': [3583], '(arrowheads,': [3584], 'upper': [3585], 'right),': [3586], 'right)': [3595], '23-week-old': [3597], '50': [3602, 3843], 'EV1L.': [3607], 'Transmission': [3608], 'electron': [3609, 3847], '14-week-old': [3615], 'Dotted': [3618], 'lines': [3619], 'thickening': [3621], 'membrane': [3624], 'panel).': [3626], 'Data': [3638, 3930], 'information:': [3639, 3931], '(D,': [3641], 'E),': [3642], 'filled': [3643, 3939], 'circles:': [3644, 3649, 3940, 3945], 'mice;': [3646, 3774, 3942], 'open': [3648, 3944], 'Each': [3652, 3948], 'circle': [3653, 3949], 'individual': [3656, 3952], 'mouse.': [3657, 3953], '(D),': [3659], 'bars': [3660], 'mean.': [3662, 3956], 'Mann–Whitney,': [3663, 3957], '**P': [3664, 3961], '<': [3665, 3668, 3959, 3962, 3965], '0.01,': [3666, 3963], '***P': [3667, 3964], '0.001.': [3669], 'Download': [3670, 3672, 3981, 3983], 'figure': [3671, 3982], 'PowerPoint': [3673, 3984], 'Click': [3674], 'here': [3675], 'expand': [3677], 'figure.': [3679, 3980], 'EV1.': [3681], 'Characterization': [3682], 'A,': [3688], 'Western': [3690], 'blot': [3691], '(A)': [3692], 'quantification': [3694, 3808, 3832], '(B)': [3695], 'medulla': [3701], 'lysates': [3702, 4021], 'remaining': [3712], 'derives': [3715], 'proximal': [3717], 'glomeruli': [3721], 'targeted': [3725], 'Ksp-Cre.': [3727], 'C.': [3728], 'descending': [3734], 'thin': [3735], 'limb': [3736, 3744], 'Henle': [3738, 3746], '(AQP1': [3739], 'thick': [3742], 'ascending': [3743], '(Tamm-Horsfall': [3747], 'expressing,': [3748, 3821], 'THP)': [3749], 'AQP1-expressing': [3769], 'panel:': [3772], 'lower': [3775], 'panel': [3776], 'mice)': [3778], 'THP-positive': [3780, 3790], 'animals,': [3784], 'mice,': [3794], 'where': [3795], 'Ksp-Cre': [3796], 'active.': [3798], 'D,': [3803], 'E.': [3804], 'Staining': [3805], '(D)': [3806], '(E)': [3809], '(CD)': [3817], '(Dolichos': [3818], 'Biflorus': [3819], 'Agglutinin': [3820], 'DBA)': [3822], 'Blinded': [3831], 'ten': [3834], 'fields': [3835], 'view': [3837], 'per': [3838], 'biological': [3839], 'sample.': [3840], 'F.': [3845], 'Scanning': [3846], 'micrographs': [3848], 'μm,': [3862], 'high': [3863], '(right):': [3865], 'G.': [3868], 'Spot': [3869], 'H,': [3874, 3935], 'I.': [3875], 'Urinary': [3876], 'rate': [3878], '(H)': [3879], 'osmolality': [3882], '(I)': [3883], 'J.': [3890], 'Scales': [3901], 'cm.': [3903], 'K.': [3904], 'weight': [3906, 3908], '(KW)-to-body': [3907], '(BW)': [3909], 'ratio': [3910], 'L.': [3917], 'collagen': [3919], 'mRNA': [3920], 'content': [3921], 'evaluated': [3922], 'qRT–PCR': [3924], '(B,': [3933], 'E,': [3934], 'I,': [3936], 'K,': [3937], 'L),': [3938], 'Bars': [3954], '*P': [3958], '0.05,': [3960], '0.001,': [3966], 'ns:': [3967], 'statistically': [3969], 'different.': [3970], 'w:': [3971], 'data': [3974], 'available': [3976], 'online': [3977], 'interacts': [3986], 'proteins,': [3989], 'functional': [3992], 'interaction': [3993], 'exists': [3994], 'between': [3995], 'obtain': [4000], 'might': [4004, 4033], 'explain': [4005], 'kidney,': [4013], 'performed': [4015], 'proteomic': [4017], 'screen': [4018], 'medulla,': [4024], 'aiming': [4025], 'endogenous': [4028], 'interactors': [4029], 'illuminate': [4034], 'function.': [4036], 'precipitates': [4039], 'ly': [4043]}, 'cited_by_api_url': 'https://api.openalex.org/works?filter=cites:W2809347302', 'counts_by_year': [{'year': 2024, 'cited_by_count': 17}, {'year': 2023, 'cited_by_count': 10}, {'year': 2022, 'cited_by_count': 16}, {'year': 2021, 'cited_by_count': 17}, {'year': 2020, 'cited_by_count': 16}, {'year': 2019, 'cited_by_count': 15}, {'year': 2018, 'cited_by_count': 1}], 'updated_date': '2024-12-24T08:52:40.577778', 'created_date': '2018-06-29'}