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Neonatal Cholestatic Jaundice as the First Symptom of a Mutation in the Hepatocyte Nuclear Factor-1β gene (HNF-1β)

✍ Scribed by Dominique Beckers; Christine Bellanné-Chantelot; Marc Maes

Book ID
113743851
Publisher
Elsevier Science
Year
2007
Tongue
English
Weight
69 KB
Volume
150
Category
Article
ISSN
1097-6833

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One form of maturity-onset diabetes of the young, Type 3 (MODY3), results from mutations in the gene coding for hepatocyte nuclear factor-1␣ (HNF-1␣), a transcription factor first described in the liver. MODY3 is characterized by a defective glucosestimulated insulin secretion. Earlier observations