MODY 1

MODY 1 or HNF4A-MODY is a form of maturity onset diabetes of the young.This transcription network plays a role in the early development of the pancreas, liver, and intestines.Diabetes (persistent hyperglycemia) typically develops by early adult years, but may not appear until later decades.Many patients with MODY 1 are treated with sulfonylureas for years before insulin is required.Many people with this condition have low levels of triglycerides, lipoprotein(a), apolipoproteins AII and CIII.

maturity onset diabetes of the youngchromosome 12transcription factorHNF1αMODY 3HNF1βMODY 5pancreasintestinesglucose transportersulfonylureastriglycerideslipoprotein(a)apolipoproteinsOrphanetglucose metabolismDiabetestype 1type 2gestationalComplicationsblood glucoseHyperglycemiaOxyhyperglycemiaHypoglycemiaWhipple's triadInsulinInsulin resistanceHyperinsulinismCongenital hyperinsulinismRabson–Mendenhall syndromePancreatic beta cell functionInsulinomaInsulitisGenetic disordersFeingold syndromeSaethre–Chotzen syndromeTietz syndromeIntracellular receptorThyroid hormone resistanceAndrogen insensitivity syndromeKennedy's diseasePHA1AD pseudohypoaldosteronismEstrogen insensitivity syndromeX-linked adrenal hypoplasia congenitaFamilial partial lipodystrophy 3SF1 XY gonadal dysgenesisBarakat syndromeTricho–rhino–phalangeal syndromeGreig cephalopolysyndactyly syndromePallister–Hall syndromeDenys–Drash syndromeDuane-radial ray syndromeMODY 7MRX 89Townes–Brocks syndromeAcrocallosal syndromeMyotonic dystrophy 2Autoimmune polyendocrine syndrome type 1Ohtahara syndromeLissencephaly X2Currarino syndromeHOXD13SPD1 synpolydactylyMODY 4Nail–patella syndromeTooth and nail syndromeAxenfeld syndrome 1POU4F3DFNA15POU3F4Posterior polymorphous corneal dystrophyFuchs' dystrophy 3Mowat–Wilson syndromePapillorenal syndromeWaardenburg syndrome 1&3MODY 9Gillespie syndromeColoboma of optic nerveCongenital hypothyroidism 2STHAG3Axenfeld syndrome 3Iridogoniodysgenesis, dominant typeLymphedema–distichiasis syndromeBamforth–Lazarus syndromeAnterior segment mesenchymal dysgenesisACD/MPVEnlarged vestibular aqueductPremature ovarian failure 3Van der Woude syndromePopliteal pterygium syndromeHyperimmunoglobulin E syndromeHolt–Oram syndromeLi–Fraumeni syndromeUlnar–mammary syndromeCampomelic dysplasiaSRY XY gonadal dysgenesisPremature ovarian failure 7Waardenburg syndrome 4cYemenite deaf-blind hypopigmentation syndromeCleidocranial dysostosisKabuki syndromePitt–Hopkins syndromeRapp–Hodgkin syndromeHay–Wells syndromeEctrodactyly–ectodermal dysplasia–cleft syndrome 3Limb–mammary syndromeTranscription coregulatorsCREBBPRubinstein–Taybi syndromeAtrichia with papular lesions