235:) in patient and family. However, Urbanova et al. found that about one quarter of Central European MODY patients are positive for islet cell autoantibodies (GABA and IA2A). Their expression is transient but highly prevalent. The autoantibodies were found in patients with delayed diabetes onset, and in times of insufficient diabetes control. The islet cell autoantibodies are absent in MODY in at least some populations (Japanese, Britons).
784:. In retrospect we can now recognize that this category covered a heterogeneous collection of disorders which included cases of dominantly inherited diabetes (the topic of this article, still called MODY today), as well as cases of what we would now call type 2 diabetes occurring in childhood or adolescence, and a few even rarer types of hyperglycemia (e.g.,
802:
Since the 1990s, as the understanding of the pathophysiology of diabetes has improved, the concept and usage of MODY have become refined and narrower. It is now used as a synonym for dominantly inherited, monogenic defects of insulin secretion occurring at any age, and no longer includes any forms of
707:
channel forms of neonatal-onset diabetes. The mouse model of MODY diabetes suggested that the reduced clearance of sulfonylureas stands behind their therapeutic success in human MODY patients, but
Urbanova et al. found that human MODY patients respond differently to the mouse model and that there was
661:
By definition, the forms of MODY are autosomal dominant, requiring only one abnormal gene to produce the disease; the severity of the disease is moderated by the presence of a second, normal allele which presumably functions normally. However, conditions involving people carrying two abnormal alleles
742:
When oral hypoglycemic agents are used in MODY, the sulfonylureas remain the oral medication of first resort. When compared to patients with type 2 diabetes, MODY patients are often more sensitive to sulphonylureas, such that a lower dose should be used to initiate treatment to avoid hypoglycaemia.
119:
MODY is the final diagnosis in 1%–2% of people initially diagnosed with diabetes. The prevalence is 70–110 per million people. 50% of first-degree relatives will inherit the same mutation, giving them a greater than 95% lifetime risk of developing MODY themselves. For this reason, correct diagnosis
681:
Homozygous mutations in the other forms have not yet been described. Those mutations for which a homozygous form has not been described may be extremely rare, may result in clinical problems not yet recognized as connected to the monogenic disorder, or may be lethal for a fetus and not result in a
771:
The term MODY dates back to 1964, when diabetes mellitus was considered to have two main forms: juvenile-onset and maturity-onset, which roughly corresponded to what we now call type 1 and type 2. MODY was originally applied to any child or young adult who had persistent, asymptomatic
208:
substitutions have been discovered. In some cases, there are significant differences in the activity of the mutant gene product that contribute to variations in the clinical features of the diabetes (such as degree of insulin deficiency or age of onset).
150:
MODY cases may make up as many as 5% of presumed type 1 and type 2 diabetes cases in a large clinic population. While the goals of diabetes management are the same no matter what type, there are two primary advantages of confirming a diagnosis of MODY.
2476:
959:
Urbanova J, Rypackova B, Prochazkova Z, Kucera P, Cerna M, Andel M, Heneberg P (2014). "Positivity for islet cell autoantibodies in patients with monogenic diabetes is associated with later diabetes onset and higher HbA1c level".
1702:
735:, and insulin injections. In many cases these goals can be achieved more easily with MODY than with ordinary types 1 and 2 diabetes. Some people with MODY may require insulin injections to achieve the same
1451:"Incidence of Childhood Diabetes in Children Aged Less than 15 Years and Its Clinical and Metabolic Characteristics at the Time of Diagnosis: Data from the Childhood Diabetes Registry of Saxony, Germany"
2469:
166:
As it occurs infrequently, many cases of MODY are initially assumed to be more common forms of diabetes: type 1 if the patient is young and not overweight, type 2 if the patient is overweight, or
1695:
2462:
1449:
Galler, Angela; Stange, Thoralf; Müller, Gabriele; Näke, Andrea; Vogel, Christian; Kapellen, Thomas; Bartelt, Heike; Kunath, Hildebrand; Koch, Rainer; Kiess, Wieland; Rothe, Ulrike (2010).
3389:
1688:
94:) involve more complex combinations of causes involving multiple genes and environmental factors, each forms of MODY are caused by changes to a single gene (monogenic). GCK-MODY (
120:
of this condition is important. Typically patients present with a strong family history of diabetes (any type) and the onset of symptoms is in the second to fifth decade.
134:
In contrast, many people with MODY have no signs or symptoms and are diagnosed either by accident, when a high glucose is discovered during testing for other reasons, or
2125:
105:
Robert
Tattersall and Stefan Fajans initially identified the phenomenon known as maturity onset diabetes of the young in a classic study published in the journal
1414:
Urbanova, J.; et al. (2015). "Half-Life of
Sulfonylureas in HNF1A and HNF4A Human MODY Patients is not Prolonged as Suggested by the Mouse Hnf1a-/- Model".
3281:
221:
Mild to moderate hyperglycemia (typically 130–250 mg/dL, or 7–14 mmol/L) discovered before 25 years of age. However, anyone under 50 can develop MODY.
670:. About 6 cases have been reported worldwide. All have required insulin treatment from shortly after birth. The condition does not seem to improve with age.
1322:
Raeder H, Johansson S, Holm PI, et al. (January 2006). "Mutations in the CEL VNTR cause a syndrome of diabetes and pancreatic exocrine dysfunction".
2644:
2885:
2715:
2586:
843:
819:
3076:
2576:
2608:
1391:
3048:
3367:
2957:
2679:
2660:
2581:
1947:
1750:
662:
have been identified. Unsurprisingly, combined (homozygous) defects of these genes are much rarer and much more severe in their effects.
3034:
2443:
1633:
1364:
1306:
2593:
1917:
1775:
1536:
481:
One of the less common forms of MODY, with some distinctive clinical features, including atrophy of the pancreas and several forms of
2454:
2397:
2047:
2684:
2273:
2603:
2571:
763:
According to data from Saxony, Germany, MODY was responsible for 2.4% of diabetes incidence in children younger than 15 years.
1858:
155:
Insulin may not be necessary and it may be possible to switch a person from insulin injections to oral agents without loss of
3193:
2618:
694:
In MODY2, oral agents are relatively ineffective, however most patients are managed conservatively through diet and exercise.
3431:
3062:
248:
Absence of obesity (although overweight or obese people can get MODY) or other problems associated with type 2 diabetes or
3486:
3455:
2163:
1874:
294:
Renal cysts, rudimentary or bicornuate uterus, vaginal aplasia, absence of the vas deferens, epidymal cysts in MODY type 5
2843:
2516:
3162:
3104:
3090:
2203:
2130:
510:
2664:
2743:
2115:
2040:
2035:
1716:
716:. The principal treatment goals for people with MODY — keeping the blood sugars as close to normal as possible ("good
239:
3219:
2689:
217:
The following characteristics suggest the possibility of a diagnosis of MODY in hyperglycemic and diabetic patients:
86:, MODY is a form of the conditions known as monogenic diabetes. While the more common types of diabetes (especially
3481:
2566:
2347:
2176:
1983:
1851:
667:
559:
3393:
3381:
3353:
2904:
2810:
1121:"Prevalence of vascular complications among patients with glucokinase mutations and prolonged, mild hyperglycemia"
422:
gene). 30%–70% of cases. Most common type of MODY in populations with
European ancestry. Tend to be responsive to
3409:
3262:
3118:
2990:
2674:
2250:
2198:
2095:
713:
708:
no consistent decrease in the clearance of sulfonylureas in randomly selected HNF1A-MODY and HNF4A-MODY patients.
261:
3214:
2669:
1097:
3299:
2433:
2171:
1978:
1644:
1258:"Role of transcription factor KLF11 and its diabetes-associated gene variants in pancreatic beta cell function"
394:
loading. Patients do not tend to get diabetes complications and do not require treatment outside of pregnancy.
285:
91:
87:
673:
MODY4: Homozygous IPF1 results in failure of the pancreas to form. Congenital absence of the pancreas, termed
566:. It is very rare with five families reported to date. It is associated with exocrine pancreatic dysfunction.
1221:"beta-cell genes and diabetes: molecular and clinical characterization of mutations in transcription factors"
666:
MODY2: Homozygous glucokinase deficiency causes severe congenital insulin deficiency resulting in persistent
2976:
2824:
2598:
2325:
2290:
785:
732:
171:
3385:
3209:
170:
if the patient is pregnant. Standard diabetes treatments (insulin for type 1 and gestational diabetes, and
3251:
3157:
2562:
2238:
2120:
2090:
2080:
1995:
1966:
1910:
868:
139:
1172:"Management and pregnancy outcomes of women with GCK-MODY enrolled in the US Monogenic Diabetes Registry"
674:
131:
and the typical signs and symptoms of diabetes: increased thirst and urination (polydipsia and polyuria).
3257:
2929:
2871:
2857:
2694:
2623:
2268:
2263:
2057:
781:
138:
of relatives of a person discovered to have diabetes. Discovery of mild hyperglycemia during a routine
697:
In MODY1 and MODY3, sulfonylureas are usually very effective, delaying the need for insulin treatment.
3425:
3397:
3276:
3235:
2943:
2829:
2489:
2428:
2387:
2320:
2310:
2305:
2193:
1960:
1745:
194:
167:
789:
2890:
2438:
2423:
2352:
2337:
2332:
2315:
2300:
2285:
2228:
2223:
2188:
2155:
2085:
1048:
Lerario, A. M.; Brito, L. P.; Mariani, B. M.; Fragoso, M. C.; Machado, M. A.; Teixeira, R. (2010).
1003:
736:
717:
156:
1026:
629:
Mutated B-lymphocyte tyrosine kinase, which is also present in pancreatic islet cells. Very rare.
2971:
2768:
2699:
2402:
1990:
1841:
1712:
1680:
1548:
1478:
1347:
985:
744:
720:"), while minimizing other vascular risk factors — are the same for all known forms of diabetes.
316:
267:
249:
107:
75:
1820:
3029:
2749:
2511:
2208:
2145:
2075:
2030:
1903:
1781:
1724:
1655:
1597:
1540:
1505:
1470:
1431:
1387:
1381:
1339:
1289:
1238:
1201:
1152:
1079:
977:
919:
888:"Maturity-onset diabetes of the young (MODY): current perspectives on diagnosis and treatment"
752:
608:
Mutations in the insulin gene. Usually associated with neonatal diabetes. Rare < 1% cases.
281:
135:
83:
71:
46:
635:
614:
593:
2639:
2485:
2183:
1808:
1587:
1579:
1532:
1462:
1450:
1423:
1331:
1279:
1269:
1228:
1191:
1183:
1142:
1132:
1069:
1061:
969:
909:
899:
777:
724:
572:
546:
519:
174:
for type 2) are often initiated before the doctor suspects a more unusual form of diabetes.
67:
3326:
3112:
3023:
2382:
2052:
1953:
1940:
1846:
1740:
1735:
1369:
MATURITY-ONSET DIABETES OF THE YOUNG, TYPE VIII, WITH EXOCRINE DYSFUNCTION; MODY8 - 609812
723:
The tools for management are similar for all forms of diabetes: blood testing, changes in
182:
The recognised forms of MODY are all due to ineffective insulin production or release by
712:
Chronic hyperglycemia due to any cause can eventually cause blood vessel damage and the
238:
Persistence of a low insulin requirement (e.g., less than 0.5 u/kg/day) past the usual "
162:
It may prompt screening of relatives and so help identify other cases in family members.
2782:
2532:
2342:
2215:
1592:
1567:
1196:
1171:
1074:
1049:
914:
887:
257:
1427:
1284:
1257:
562:
has been associated with a form of diabetes that has been characterized as "MODY8" by
536:
has been associated with a form of diabetes that has been characterized as "MODY7" by
3475:
3132:
2754:
2359:
2280:
2258:
1803:
1794:
773:
319:
fashion, and most patients therefore have other members of the family with diabetes;
128:
51:
1552:
1065:
2364:
2105:
2100:
1815:
1482:
1351:
1170:
Dickens LT, Letourneau LR, Sanyoura M, Greeley SA, Philipson LH, Naylor RN (2019).
989:
793:
700:
690:
In some forms of MODY, standard treatment is appropriate, though exceptions occur:
645:
458:
423:
253:
228:
1523:
Tattersall, R (1998). "Maturity-onset diabetes of the young: A clinical history".
17:
1649:
1233:
1220:
2407:
2135:
1973:
1119:
Steele AM, Shields BM, Wensley KJ, Colclough K, Ellard S, Hattersley AT (2014).
381:
232:
201:
937:
799:
The current usage of the term MODY dates from a case report published in 1974.
677:, involves deficiency of both endocrine and exocrine functions of the pancreas.
390:
gene. 30%–70% cases. Mild fasting hyperglycemia throughout life. Small rise on
3004:
2067:
1879:
1660:
1187:
624:
454:
320:
205:
1137:
1120:
204:
gene. For each form of MODY, multiple specific mutations involving different
3450:
2110:
1884:
1274:
1256:
Neve B, Fernandez-Zapico ME, Ashkenazi-Katalan V, et al. (March 2005).
748:
747:
than those with ordinary type 2 diabetes (for whom insulin sensitizers like
650:
Mutated anchor protein in the insulin signalling pathway. Newly discovered.
186:
143:
1601:
1474:
1435:
1343:
1293:
1242:
1205:
1156:
1083:
981:
923:
1544:
1509:
1496:
Tattersall RB (1974). "Mild familial diabetes with dominant inheritance".
1050:"A missense TCF1 mutation in a patient with MODY-3 and liver adenomatosis"
739:
that another person may attain with careful eating or an oral medication.
2140:
1926:
1537:
10.1002/(SICI)1096-9136(199801)15:1<11::AID-DIA561>3.0.CO;2-0
904:
728:
587:
Pax4 is a transcription factor. MODY 9 is a very rare medical condition.
450:
419:
190:
183:
1832:
1147:
603:
391:
79:
1625:
1583:
1466:
973:
3245:
3240:
2865:
2851:
2796:
2776:
2613:
2023:
2020:
2017:
2014:
2011:
2008:
1768:
1765:
1762:
1759:
1756:
1753:
495:
482:
467:
432:
400:
370:
346:
274:
99:
95:
1335:
453:(Pdx1) gene. < 1% cases. Associated with pancreatic agenesis in
3361:
3270:
3126:
3098:
3084:
3070:
3056:
3042:
2837:
2804:
533:
509:
Mutations of the gene for the transcription factor referred to as
486:
415:
361:
1674:
1219:
Frayling, TM; Evans, JC; Bulman, MP; Pearson, E (February 2001).
3375:
3347:
3151:
2998:
2984:
2965:
2951:
2937:
2923:
2898:
2879:
2818:
2790:
2762:
2392:
1638:
1368:
1310:
848:
National
Institute of Diabetes and Digestive and Kidney Diseases
824:
National
Institute of Diabetes and Digestive and Kidney Diseases
640:
619:
598:
582:
577:
563:
551:
537:
525:
501:
473:
447:
438:
406:
376:
352:
332:
299:
197:
2458:
1899:
1684:
1311:
MATURITY-ONSET DIABETES OF THE YOUNG, TYPE VII; MODY7 - 610508
892:
Diabetes, Metabolic
Syndrome and Obesity: Targets and Therapy
844:"Monogenic Diabetes (Neonatal Diabetes Mellitus & MODY)"
820:"Monogenic Diabetes (Neonatal Diabetes Mellitus & MODY)"
1895:
869:"'Maturity Onset Diabetes of the Young (MODY) | Dr Mohans"
224:
A first-degree relative with a similar degree of diabetes.
898:. Diabetes Metabolic Syndrome & Obesity: 1047–1056.
291:
Liver adenoma or hepatocellular carcinoma in MODY type 3
743:
Patients with MODY less often suffer from obesity and
123:
There are two general types of clinical presentation.
1615:
3442:
3417:
3408:
3339:
3319:
3312:
3292:
3228:
3202:
3186:
3177:
3143:
3015:
2915:
2735:
2728:
2708:
2653:
2632:
2554:
2545:
2525:
2504:
2497:
2416:
2373:
2249:
2154:
2066:
1933:
1867:
1831:
1792:
1723:
1619:
1568:"Diagnosis and Classification of Diabetes Mellitus"
302:testing available through commercial laboratories.
45:
37:
32:
3390:Ectrodactyly–ectodermal dysplasia–cleft syndrome 3
2126:Metabolic Score for Insulin Resistance (METS-IR)
298:The diagnosis of MODY is confirmed by specific
1677:. Information about genetic forms of diabetes.
3282:Yemenite deaf-blind hypopigmentation syndrome
2470:
1911:
1696:
1386:. Oxford University Press US. pp. 184–.
755:are often preferred over the sulfonylureas).
323:differs between the types (from 40% to 90%).
8:
792:). Many of these patients were treated with
33:Maturity-onset diabetes of the young (MODY)
3414:
3316:
3183:
2732:
2551:
2501:
2477:
2463:
2455:
1918:
1904:
1896:
1703:
1689:
1681:
1616:
1380:Dhavendra Kumar; D. J. Weatherall (2008).
867:Chennai, India, Dr. V Mohan (2020-07-15).
513:. Very rare: 5 families reported to date.
360:Due to a loss-of-function mutation in the
29:
1868:Other pancreatic disorders and conditions
1591:
1283:
1273:
1232:
1195:
1146:
1136:
1073:
913:
903:
2886:Posterior polymorphous corneal dystrophy
2716:Autoimmune polyendocrine syndrome type 1
325:
227:Absence of positive antibodies or other
3077:Anterior segment mesenchymal dysgenesis
1566:American Diabetes, Association (2009).
811:
714:microvascular complications of diabetes
386:Due to any of several mutations in the
127:Some forms of MODY produce significant
277:disease in patient or close relatives.
200:. One form is due to mutations of the
2609:X-linked adrenal hypoplasia congenita
873:Dr Mohan's Diabetes Center in Chennai
245:Normal insulin levels (e.g. 2.6-24.9)
7:
2661:Greig cephalopolysyndactyly syndrome
2168:Metabolic assessment and monitoring
288:with onset before six months of age.
60:Maturity-onset diabetes of the young
3035:Iridogoniodysgenesis, dominant type
2444:Notable people with type 1 diabetes
1956:(SIDD, SIRD, MOD and MARD clusters)
1365:Online Mendelian Inheritance in Man
1307:Online Mendelian Inheritance in Man
426:. Low renal threshold for glucose.
25:
2398:International Diabetes Federation
1428:10.2174/1381612821666151008124036
1098:Renal Cysts and Diabetes Syndrome
796:with varying degrees of success.
3049:Lymphedema–distichiasis syndrome
2645:Tricho–rhino–phalangeal syndrome
2619:Familial partial lipodystrophy 3
2274:Hyperosmolar hyperglycemic state
3313:(0) Other transcription factors
2604:Estrogen insensitivity syndrome
2572:Androgen insensitivity syndrome
1455:Hormone Research in Paediatrics
1066:10.1590/S1807-59322010001000024
146:is particularly characteristic.
3194:Hyperimmunoglobulin E syndrome
2599:PHA1AD pseudohypoaldosteronism
1383:Genomics and clinical medicine
1:
3456:Atrichia with papular lesions
2333:Diabetes-related skin disease
1875:Pancreatic beta cell function
1416:Current Pharmaceutical Design
506:neurogenic differentiation 1
478:hepatocyte nuclear factor 1β
411:hepatocyte nuclear factor 1α
357:hepatocyte nuclear factor 4α
189:. Several of the defects are
102:) are the most common forms.
3163:Popliteal pterygium syndrome
3105:Enlarged vestibular aqueduct
2944:Waardenburg syndrome 1&3
2729:(3) Helix-turn-helix domains
2488:relating to deficiencies of
2131:Homeostatic model assessment
1262:Proc. Natl. Acad. Sci. U.S.A
1234:10.2337/diabetes.50.2007.S94
511:neurogenic differentiation 1
3263:Premature ovarian failure 7
3119:Premature ovarian failure 3
2991:Congenital hypothyroidism 2
2116:Noninvasive glucose monitor
556:Bile salt dependent lipase
66:) refers to any of several
3503:
3410:Transcription coregulators
3180:with minor groove contacts
2567:Thyroid hormone resistance
2348:Diabetic cheiroarthropathy
2177:Ambulatory glucose profile
1984:Impaired glucose tolerance
1859:Rabson–Mendenhall syndrome
1852:Congenital hyperinsulinism
668:neonatal diabetes mellitus
443:insulin promoter factor-1
74:caused by mutations in an
3432:Rubinstein–Taybi syndrome
3258:SRY XY gonadal dysgenesis
3063:Bamforth–Lazarus syndrome
2675:Duane-radial ray syndrome
2624:SF1 XY gonadal dysgenesis
2096:Postprandial glucose test
1227:. 50 Suppl (1): S94-100.
1188:10.1007/s00592-018-1267-z
262:polycystic ovary syndrome
3300:Cleidocranial dysostosis
2517:Saethre–Chotzen syndrome
2434:Epidemiology of diabetes
2172:Blood glucose monitoring
2048:Type 3c (pancreatogenic)
1979:Impaired fasting glucose
1138:10.1001/jama.2013.283980
733:oral hypoglycemic agents
315:MODY is inherited in an
172:oral hypoglycemic agents
3277:Waardenburg syndrome 4c
2977:Coloboma of optic nerve
2825:Tooth and nail syndrome
2665:Pallister–Hall syndrome
2291:Neuropathic arthropathy
1275:10.1073/pnas.0409177102
1005:Maturity Onset Diabetes
776:without progression to
530:Kruppel-like factor 11
82:production. Along with
76:autosomal dominant gene
3220:Ulnar–mammary syndrome
3178:(4) β-Scaffold factors
3158:Van der Woude syndrome
2690:Townes–Brocks syndrome
2563:Intracellular receptor
2239:Gastric bypass surgery
2121:Insulin tolerance test
2091:Glucose tolerance test
1996:Ketosis-prone diabetes
1967:Diabetes and pregnancy
786:mitochondrial diabetes
703:are effective in the K
140:glucose tolerance test
3394:Limb–mammary syndrome
3382:Rapp–Hodgkin syndrome
3354:Pitt–Hopkins syndrome
2930:Papillorenal syndrome
2905:Mowat–Wilson syndrome
2811:Nail–patella syndrome
2695:Acrocallosal syndrome
3487:Congenital disorders
3236:Campomelic dysplasia
3215:Li–Fraumeni syndrome
2700:Myotonic dystrophy 2
2670:Denys–Drash syndrome
2490:transcription factor
2429:Glossary of diabetes
2388:Open Insulin Project
2224:Embryonic stem cells
1961:Gestational diabetes
1578:(Suppl 1): S62–S67.
905:10.2147/DMSO.S179793
489:gene. 5%–10% cases.
457:and occasionally in
364:gene. 5%–10% cases.
270:very rarely happens.
195:transcription factor
168:gestational diabetes
3030:Axenfeld syndrome 3
2844:Axenfeld syndrome 1
2783:SPD1 synpolydactyly
2548:DNA-binding domains
2439:History of diabetes
2424:Outline of diabetes
2353:Diabetic foot ulcer
2338:Diabetic dermopathy
2297:Organs in diabetes
2229:Artificial pancreas
2189:Diabetes medication
2086:Glycated hemoglobin
675:pancreatic agenesis
485:disease. Defect in
3386:Hay–Wells syndrome
3210:Holt–Oram syndrome
2972:Gillespie syndrome
2891:Fuchs' dystrophy 3
2769:Currarino syndrome
2403:World Diabetes Day
1991:Insulin resistance
1842:Insulin resistance
1717:glucose metabolism
1176:Acta Diabetologica
886:Urakami T (2019).
753:thiazolidinediones
745:insulin resistance
317:autosomal dominant
268:Insulin resistance
250:metabolic syndrome
115:Signs and symptoms
98:) and HNF1A-MODY (
41:Monogenic diabetes
18:Monogenic diabetes
3482:Types of diabetes
3469:
3468:
3465:
3464:
3335:
3334:
3308:
3307:
3173:
3172:
2750:Ohtahara syndrome
2724:
2723:
2594:Kennedy's disease
2541:
2540:
2512:Feingold syndrome
2498:(1) Basic domains
2486:Genetic disorders
2452:
2451:
2146:Disposition index
2076:Blood sugar level
1946:Type 1 diabetes (
1893:
1892:
1782:Template:Diabetes
1670:
1669:
1584:10.2337/dc09-S062
1525:Diabetic Medicine
1467:10.1159/000303141
1422:(39): 5736–5748.
1393:978-0-19-518813-4
1060:(10): 1059–1060.
1029:(Report). Harvard
974:10.1111/dme.12314
962:Diabetic Medicine
803:type 2 diabetes.
654:
653:
446:Mutations of the
414:Mutations of the
282:neonatal diabetes
84:neonatal diabetes
72:diabetes mellitus
57:
56:
27:Medical condition
16:(Redirected from
3494:
3415:
3317:
3184:
2755:Lissencephaly X2
2733:
2640:Barakat syndrome
2552:
2502:
2479:
2472:
2465:
2456:
2184:Diet in diabetes
1920:
1913:
1906:
1897:
1809:Oxyhyperglycemia
1705:
1698:
1691:
1682:
1617:
1606:
1605:
1595:
1563:
1557:
1556:
1520:
1514:
1513:
1504:(170): 339–357.
1493:
1487:
1486:
1446:
1440:
1439:
1411:
1405:
1404:
1402:
1400:
1377:
1371:
1362:
1356:
1355:
1319:
1313:
1304:
1298:
1297:
1287:
1277:
1253:
1247:
1246:
1236:
1216:
1210:
1209:
1199:
1167:
1161:
1160:
1150:
1140:
1116:
1110:
1109:
1107:
1105:
1094:
1088:
1087:
1077:
1045:
1039:
1038:
1036:
1034:
1023:
1017:
1016:
1015:
1013:
1000:
994:
993:
956:
950:
949:
947:
945:
934:
928:
927:
917:
907:
883:
877:
876:
864:
858:
857:
855:
854:
840:
834:
833:
831:
830:
816:
778:diabetic ketosis
737:glycemic control
718:glycemic control
326:
157:glycemic control
30:
21:
3502:
3501:
3497:
3496:
3495:
3493:
3492:
3491:
3472:
3471:
3470:
3461:
3438:
3404:
3331:
3327:Kabuki syndrome
3304:
3288:
3224:
3198:
3179:
3169:
3139:
3011:
2911:
2720:
2704:
2649:
2628:
2547:
2546:(2) Zinc finger
2537:
2521:
2493:
2492:or coregulators
2483:
2453:
2448:
2412:
2383:T1International
2375:
2374:Advocacy &
2369:
2245:
2194:Insulin therapy
2150:
2062:
1929:
1924:
1894:
1889:
1863:
1847:Hyperinsulinism
1827:
1821:Whipple's triad
1788:
1719:
1711:Disease of the
1709:
1671:
1666:
1665:
1628:
1614:
1609:
1565:
1564:
1560:
1522:
1521:
1517:
1495:
1494:
1490:
1448:
1447:
1443:
1413:
1412:
1408:
1398:
1396:
1394:
1379:
1378:
1374:
1363:
1359:
1321:
1320:
1316:
1305:
1301:
1268:(13): 4807–12.
1255:
1254:
1250:
1218:
1217:
1213:
1169:
1168:
1164:
1118:
1117:
1113:
1103:
1101:
1096:
1095:
1091:
1047:
1046:
1042:
1032:
1030:
1025:
1024:
1020:
1011:
1009:
1002:
1001:
997:
958:
957:
953:
943:
941:
936:
935:
931:
885:
884:
880:
866:
865:
861:
852:
850:
842:
841:
837:
828:
826:
818:
817:
813:
809:
769:
761:
706:
688:
659:
313:
308:
286:type 1 diabetes
215:
180:
178:Pathophysiology
117:
28:
23:
22:
15:
12:
11:
5:
3500:
3498:
3490:
3489:
3484:
3474:
3473:
3467:
3466:
3463:
3462:
3460:
3459:
3446:
3444:
3440:
3439:
3437:
3436:
3435:
3434:
3421:
3419:
3412:
3406:
3405:
3403:
3402:
3401:
3400:
3372:
3371:
3370:
3358:
3357:
3356:
3343:
3341:
3337:
3336:
3333:
3332:
3330:
3329:
3323:
3321:
3314:
3310:
3309:
3306:
3305:
3303:
3302:
3296:
3294:
3290:
3289:
3287:
3286:
3285:
3284:
3279:
3267:
3266:
3265:
3260:
3248:
3243:
3238:
3232:
3230:
3226:
3225:
3223:
3222:
3217:
3212:
3206:
3204:
3200:
3199:
3197:
3196:
3190:
3188:
3181:
3175:
3174:
3171:
3170:
3168:
3167:
3166:
3165:
3160:
3147:
3145:
3141:
3140:
3138:
3137:
3136:
3135:
3123:
3122:
3121:
3109:
3108:
3107:
3095:
3094:
3093:
3081:
3080:
3079:
3067:
3066:
3065:
3053:
3052:
3051:
3039:
3038:
3037:
3032:
3019:
3017:
3013:
3012:
3010:
3009:
3008:
3007:
2995:
2994:
2993:
2981:
2980:
2979:
2974:
2962:
2961:
2960:
2948:
2947:
2946:
2934:
2933:
2932:
2919:
2917:
2913:
2912:
2910:
2909:
2908:
2907:
2895:
2894:
2893:
2888:
2876:
2875:
2874:
2862:
2861:
2860:
2848:
2847:
2846:
2834:
2833:
2832:
2827:
2815:
2814:
2813:
2801:
2800:
2799:
2787:
2786:
2785:
2773:
2772:
2771:
2759:
2758:
2757:
2752:
2739:
2737:
2730:
2726:
2725:
2722:
2721:
2719:
2718:
2712:
2710:
2706:
2705:
2703:
2702:
2697:
2692:
2687:
2682:
2677:
2672:
2667:
2657:
2655:
2651:
2650:
2648:
2647:
2642:
2636:
2634:
2630:
2629:
2627:
2626:
2621:
2616:
2611:
2606:
2601:
2596:
2591:
2590:
2589:
2584:
2579:
2569:
2558:
2556:
2549:
2543:
2542:
2539:
2538:
2536:
2535:
2533:Tietz syndrome
2529:
2527:
2523:
2522:
2520:
2519:
2514:
2508:
2506:
2499:
2495:
2494:
2484:
2482:
2481:
2474:
2467:
2459:
2450:
2449:
2447:
2446:
2441:
2436:
2431:
2426:
2420:
2418:
2414:
2413:
2411:
2410:
2405:
2400:
2395:
2390:
2385:
2379:
2377:
2371:
2370:
2368:
2367:
2362:
2357:
2356:
2355:
2350:
2345:
2343:Diabetic bulla
2340:
2330:
2329:
2328:
2323:
2318:
2313:
2308:
2303:
2295:
2294:
2293:
2288:
2278:
2277:
2276:
2271:
2266:
2259:Diabetic comas
2255:
2253:
2247:
2246:
2244:
2243:
2242:
2241:
2233:
2232:
2231:
2226:
2218:
2216:Diabetic shoes
2213:
2212:
2211:
2206:
2201:
2191:
2186:
2181:
2180:
2179:
2174:
2166:
2160:
2158:
2152:
2151:
2149:
2148:
2143:
2138:
2133:
2128:
2123:
2118:
2113:
2108:
2103:
2098:
2093:
2088:
2083:
2078:
2072:
2070:
2064:
2063:
2061:
2060:
2055:
2050:
2045:
2044:
2043:
2038:
2028:
2027:
2026:
1999:
1993:
1988:
1987:
1986:
1981:
1971:
1970:
1969:
1957:
1951:
1944:
1943:(SAID cluster)
1937:
1935:
1931:
1930:
1925:
1923:
1922:
1915:
1908:
1900:
1891:
1890:
1888:
1887:
1882:
1877:
1871:
1869:
1865:
1864:
1862:
1861:
1856:
1855:
1854:
1844:
1838:
1836:
1829:
1828:
1826:
1825:
1824:
1823:
1813:
1812:
1811:
1800:
1798:
1790:
1789:
1787:
1786:
1785:
1784:
1773:
1772:
1771:
1748:
1743:
1738:
1729:
1727:
1721:
1720:
1710:
1708:
1707:
1700:
1693:
1685:
1679:
1678:
1675:Diabetes Genes
1668:
1667:
1664:
1663:
1652:
1641:
1629:
1624:
1623:
1621:
1620:Classification
1613:
1612:External links
1610:
1608:
1607:
1558:
1515:
1488:
1441:
1406:
1392:
1372:
1357:
1336:10.1038/ng1708
1314:
1299:
1248:
1211:
1182:(3): 405–411.
1162:
1111:
1089:
1040:
1018:
995:
951:
929:
878:
859:
835:
810:
808:
805:
790:mutant insulin
768:
765:
760:
757:
710:
709:
704:
698:
695:
687:
684:
682:viable child.
679:
678:
671:
658:
655:
652:
651:
648:
643:
638:
631:
630:
627:
622:
617:
610:
609:
606:
601:
596:
589:
588:
585:
580:
575:
568:
567:
557:
554:
549:
542:
541:
531:
528:
523:
515:
514:
507:
504:
499:
494:NEUROD1-MODY (
491:
490:
479:
476:
471:
463:
462:
444:
441:
436:
428:
427:
412:
409:
404:
396:
395:
384:
379:
374:
366:
365:
358:
355:
350:
342:
341:
338:
335:
330:
312:
309:
307:
306:Classification
304:
296:
295:
292:
289:
284:, or apparent
280:Non-transient
278:
271:
265:
258:hyperlipidemia
246:
243:
236:
225:
222:
214:
211:
179:
176:
164:
163:
160:
148:
147:
132:
116:
113:
55:
54:
49:
43:
42:
39:
35:
34:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
3499:
3488:
3485:
3483:
3480:
3479:
3477:
3457:
3453:
3452:
3448:
3447:
3445:
3441:
3433:
3430:
3429:
3428:
3427:
3423:
3422:
3420:
3416:
3413:
3411:
3407:
3399:
3395:
3391:
3387:
3383:
3380:
3379:
3378:
3377:
3373:
3369:
3366:
3365:
3364:
3363:
3359:
3355:
3352:
3351:
3350:
3349:
3345:
3344:
3342:
3338:
3328:
3325:
3324:
3322:
3318:
3315:
3311:
3301:
3298:
3297:
3295:
3291:
3283:
3280:
3278:
3275:
3274:
3273:
3272:
3268:
3264:
3261:
3259:
3256:
3255:
3254:
3253:
3249:
3247:
3244:
3242:
3239:
3237:
3234:
3233:
3231:
3227:
3221:
3218:
3216:
3213:
3211:
3208:
3207:
3205:
3201:
3195:
3192:
3191:
3189:
3185:
3182:
3176:
3164:
3161:
3159:
3156:
3155:
3154:
3153:
3149:
3148:
3146:
3142:
3134:
3131:
3130:
3129:
3128:
3124:
3120:
3117:
3116:
3115:
3114:
3110:
3106:
3103:
3102:
3101:
3100:
3096:
3092:
3089:
3088:
3087:
3086:
3082:
3078:
3075:
3074:
3073:
3072:
3068:
3064:
3061:
3060:
3059:
3058:
3054:
3050:
3047:
3046:
3045:
3044:
3040:
3036:
3033:
3031:
3028:
3027:
3026:
3025:
3021:
3020:
3018:
3014:
3006:
3003:
3002:
3001:
3000:
2996:
2992:
2989:
2988:
2987:
2986:
2982:
2978:
2975:
2973:
2970:
2969:
2968:
2967:
2963:
2959:
2956:
2955:
2954:
2953:
2949:
2945:
2942:
2941:
2940:
2939:
2935:
2931:
2928:
2927:
2926:
2925:
2921:
2920:
2918:
2914:
2906:
2903:
2902:
2901:
2900:
2896:
2892:
2889:
2887:
2884:
2883:
2882:
2881:
2877:
2873:
2870:
2869:
2868:
2867:
2863:
2859:
2856:
2855:
2854:
2853:
2849:
2845:
2842:
2841:
2840:
2839:
2835:
2831:
2828:
2826:
2823:
2822:
2821:
2820:
2816:
2812:
2809:
2808:
2807:
2806:
2802:
2798:
2795:
2794:
2793:
2792:
2788:
2784:
2781:
2780:
2779:
2778:
2774:
2770:
2767:
2766:
2765:
2764:
2760:
2756:
2753:
2751:
2748:
2747:
2746:
2745:
2741:
2740:
2738:
2734:
2731:
2727:
2717:
2714:
2713:
2711:
2707:
2701:
2698:
2696:
2693:
2691:
2688:
2686:
2683:
2681:
2678:
2676:
2673:
2671:
2668:
2666:
2662:
2659:
2658:
2656:
2652:
2646:
2643:
2641:
2638:
2637:
2635:
2631:
2625:
2622:
2620:
2617:
2615:
2612:
2610:
2607:
2605:
2602:
2600:
2597:
2595:
2592:
2588:
2585:
2583:
2580:
2578:
2575:
2574:
2573:
2570:
2568:
2564:
2560:
2559:
2557:
2553:
2550:
2544:
2534:
2531:
2530:
2528:
2524:
2518:
2515:
2513:
2510:
2509:
2507:
2503:
2500:
2496:
2491:
2487:
2480:
2475:
2473:
2468:
2466:
2461:
2460:
2457:
2445:
2442:
2440:
2437:
2435:
2432:
2430:
2427:
2425:
2422:
2421:
2419:
2415:
2409:
2406:
2404:
2401:
2399:
2396:
2394:
2391:
2389:
2386:
2384:
2381:
2380:
2378:
2376:Organizations
2372:
2366:
2363:
2361:
2360:Hyperglycemia
2358:
2354:
2351:
2349:
2346:
2344:
2341:
2339:
2336:
2335:
2334:
2331:
2327:
2324:
2322:
2319:
2317:
2314:
2312:
2309:
2307:
2304:
2302:
2301:Blood vessels
2299:
2298:
2296:
2292:
2289:
2287:
2284:
2283:
2282:
2281:Diabetic foot
2279:
2275:
2272:
2270:
2267:
2265:
2262:
2261:
2260:
2257:
2256:
2254:
2252:
2251:Complications
2248:
2240:
2237:
2236:
2234:
2230:
2227:
2225:
2222:
2221:
2219:
2217:
2214:
2210:
2207:
2205:
2202:
2200:
2197:
2196:
2195:
2192:
2190:
2187:
2185:
2182:
2178:
2175:
2173:
2170:
2169:
2167:
2165:
2162:
2161:
2159:
2157:
2153:
2147:
2144:
2142:
2139:
2137:
2134:
2132:
2129:
2127:
2124:
2122:
2119:
2117:
2114:
2112:
2109:
2107:
2104:
2102:
2099:
2097:
2094:
2092:
2089:
2087:
2084:
2082:
2079:
2077:
2074:
2073:
2071:
2069:
2065:
2059:
2056:
2054:
2051:
2049:
2046:
2042:
2039:
2037:
2034:
2033:
2032:
2029:
2025:
2022:
2019:
2016:
2013:
2010:
2007:
2006:
2004:
2000:
1997:
1994:
1992:
1989:
1985:
1982:
1980:
1977:
1976:
1975:
1972:
1968:
1965:
1964:
1962:
1958:
1955:
1952:
1949:
1945:
1942:
1939:
1938:
1936:
1932:
1928:
1921:
1916:
1914:
1909:
1907:
1902:
1901:
1898:
1886:
1883:
1881:
1878:
1876:
1873:
1872:
1870:
1866:
1860:
1857:
1853:
1850:
1849:
1848:
1845:
1843:
1840:
1839:
1837:
1834:
1830:
1822:
1819:
1818:
1817:
1814:
1810:
1807:
1806:
1805:
1804:Hyperglycemia
1802:
1801:
1799:
1796:
1795:blood glucose
1791:
1783:
1779:
1778:
1777:
1776:Complications
1774:
1770:
1767:
1764:
1761:
1758:
1755:
1752:
1749:
1747:
1744:
1742:
1739:
1737:
1734:
1733:
1731:
1730:
1728:
1726:
1722:
1718:
1714:
1706:
1701:
1699:
1694:
1692:
1687:
1686:
1683:
1676:
1673:
1672:
1662:
1658:
1657:
1653:
1651:
1647:
1646:
1642:
1640:
1636:
1635:
1631:
1630:
1627:
1622:
1618:
1611:
1603:
1599:
1594:
1589:
1585:
1581:
1577:
1573:
1572:Diabetes Care
1569:
1562:
1559:
1554:
1550:
1546:
1542:
1538:
1534:
1530:
1526:
1519:
1516:
1511:
1507:
1503:
1499:
1492:
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774:hyperglycemia
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459:heterozygotes
456:
452:
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429:
425:
424:sulfonylureas
421:
417:
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337:Gene/protein
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129:hyperglycemia
126:
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52:Endocrinology
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2365:Hypoglycemia
2269:Ketoacidosis
2264:Hypoglycemia
2204:conventional
2106:Glucose test
2101:Fructosamine
2002:
1816:Hypoglycemia
1654:
1643:
1632:
1575:
1571:
1561:
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1382:
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847:
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782:ketoacidosis
770:
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759:Epidemiology
741:
722:
711:
689:
680:
660:
518:KLF11-MODY (
466:HNF1B-MODY (
399:HNF1A-MODY (
387:
345:HNF4A-MODY (
340:Description
314:
311:Heterozygous
297:
254:hypertension
229:autoimmunity
216:
181:
165:
149:
122:
118:
106:
104:
63:
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2408:Diabetes UK
2136:SPINA-GBeta
2068:Blood tests
1974:Prediabetes
1746:gestational
1531:(1): 11–4.
1148:10871/15378
1033:January 23,
727:, physical
634:AAPL1-MODY
455:homozygotes
431:PDX1-MODY (
382:glucokinase
233:thyroiditis
202:glucokinase
78:disrupting
38:Other names
3476:Categories
2164:Prevention
2156:Management
2081:Biomarkers
1880:Insulinoma
1656:DiseasesDB
1324:Nat. Genet
853:2023-02-10
829:2023-02-01
807:References
657:Homozygous
571:PAX4-MODY
487:HNF-1 beta
369:GCK-MODY (
321:penetrance
206:amino acid
187:beta cells
184:pancreatic
68:hereditary
3340:Ungrouped
2209:pulsatile
2199:intensive
2111:C-peptide
2041:Permanent
2036:Transient
2001:Type 3a (
1885:Insulitis
1835:disorders
1793:Abnormal
749:metformin
686:Treatment
613:BLK-MODY
592:INS-MODY
545:CEL-MODY
242:" period.
240:honeymoon
213:Diagnosis
191:mutations
144:pregnancy
136:screening
111:in 1975.
70:forms of
47:Specialty
2141:SPINA-GR
2031:Neonatal
1959:Type 4 (
1950:cluster)
1927:Diabetes
1725:Diabetes
1713:pancreas
1602:19118289
1553:31690226
1475:20516654
1436:26446475
1367:(OMIM):
1344:16369531
1309:(OMIM):
1294:15774581
1243:11272211
1225:Diabetes
1206:30535721
1157:24430320
1100:(Report)
1084:21120312
982:24102923
940:(Report)
924:31360071
729:exercise
451:homeobox
420:homeobox
418:gene (a
108:Diabetes
3091:ACD/MPV
1833:Insulin
1650:D003924
1593:2613584
1545:9472858
1510:4212169
1498:Q J Med
1483:1313999
1399:12 June
1352:8338877
1197:6468988
1104:May 19,
1075:2972616
1054:Clinics
1012:Jan 21,
990:1867195
944:Jan 25,
915:6625604
767:History
751:or the
636:MODY 14
615:MODY 11
594:MODY 10
392:glucose
273:Cystic
252:(e.g.,
231:(e.g.,
80:insulin
3426:CREBBP
3246:MODY 5
3241:MODY 3
3005:STHAG3
2958:MODY 9
2866:POU3F4
2858:DFNA15
2852:POU4F3
2797:MODY 4
2777:HOXD13
2685:MRX 89
2680:MODY 7
2614:MODY 1
2321:Retina
2316:Nerves
2311:Kidney
2306:Muscle
2235:Other
2053:Type 3
2009:Type 1
1954:Type 2
1941:Type 1
1797:levels
1741:type 2
1736:type 1
1732:Types
1639:606391
1600:
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912:
641:616511
620:613375
599:613370
578:612225
573:MODY 9
552:609812
547:MODY 8
526:610508
520:MODY 7
502:606394
496:MODY 6
474:137920
468:MODY 5
439:606392
433:MODY 4
407:600496
401:MODY 3
377:125851
371:MODY 2
353:125850
347:MODY 1
275:kidney
100:MODY 3
96:MODY 2
92:type 2
88:type 1
3368:TNDM1
3362:ZFP57
3271:SOX10
3127:FOXP3
3113:FOXL2
3099:FOXI1
3085:FOXF1
3071:FOXE3
3057:FOXE1
3043:FOXC2
3024:FOXC1
2872:DFNX2
2838:PITX2
2805:LMX1B
2417:Other
2326:Heart
2286:ulcer
2220:Cure
1998:(KPD)
1934:Types
1549:S2CID
1479:S2CID
1348:S2CID
986:S2CID
646:APPL1
534:KLF11
483:renal
416:HNF1α
362:HNF4α
329:Type
198:genes
3398:OFC8
3376:TP63
3348:TCF4
3293:4.11
3152:IRF6
3133:IPEX
2999:PAX9
2985:PAX8
2966:PAX6
2952:PAX4
2938:PAX3
2924:PAX2
2899:ZEB2
2880:ZEB1
2830:OFC5
2819:MSX1
2791:PDX1
2763:MNX1
2587:CAIS
2582:MAIS
2577:PAIS
2393:JDRF
2058:MIDD
2003:MODY
1948:LADA
1780:See
1751:MODY
1715:and
1661:8330
1645:MeSH
1634:OMIM
1598:PMID
1541:PMID
1506:PMID
1471:PMID
1432:PMID
1401:2010
1388:ISBN
1340:PMID
1290:PMID
1239:PMID
1202:PMID
1153:PMID
1125:JAMA
1106:2011
1080:PMID
1035:2010
1027:MODY
1014:2010
978:PMID
946:2010
938:MODY
920:PMID
725:diet
583:PAX4
564:OMIM
538:OMIM
448:IPF1
333:OMIM
300:gene
142:for
90:and
64:MODY
3320:0.6
3252:SF1
3229:4.7
3203:4.3
3187:4.2
3144:3.5
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2744:ARX
2736:3.1
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1588:PMC
1580:doi
1533:doi
1463:doi
1424:doi
1332:doi
1280:PMC
1270:doi
1266:102
1229:doi
1192:PMC
1184:doi
1143:hdl
1133:doi
1129:311
1070:PMC
1062:doi
970:doi
910:PMC
900:doi
788:or
780:or
705:ATP
625:BLK
604:INS
560:CEL
388:GCK
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