MODY 1 is caused by mutations in
Correct Answer: HNF-4 alpha
Description: More than 10 different variants of MODY, caused by mutations in genes encoding islet-enriched transcription factors or glucokinase, are transmitted as autosomal dominant disorders.MODY 1, MODY 3, and MODY 5 are caused by mutations in the hepatocyte nuclear transcription factor (HNF) 4a, HNF-1a, and HNF-1b, respectively. As their names imply, these transcription factors are expressed in the liver but also in other tissues, including the pancreatic islets and kidney. These factors most likely affect islet development or the expression of genes impoant in glucose-stimulated insulin secretion or the maintenance of beta cell mass.Genetic defects of beta cell development or function characterized by mutations in (III A)Hepatocyte nuclear transcription factor (HNF) 4a (MODY 1)Glucokinase (MODY 2)HNF-1a (MODY 3)Insulin promoter factor-1 (IPF-1; MODY 4)HNF-1b (MODY 5)NeuroD1 (MODY 6)Mitochondrial DNASubunits of ATP-sensitive potassium channelProinsulin or insulinOther pancreatic islet regulators/proteins such as KLF11, PAX4, BLK, GATA4, GATA6, SLC2A2 (GLUT2), RFX6, GLIS3Ref: Harrison 19e pg: 2406
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