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Winter WE. Molecular and biochemical analysis of the MODY syndromes. Pediatr Diabetes. 2000;1(2):88-117doi: 10.1034/j.1399-5448.2000.010206.x.
Winter, W. E. (2000). Molecular and biochemical analysis of the MODY syndromes. Pediatric diabetes, 1(2), 88-117. https://doi.org/10.1034/j.1399-5448.2000.010206.x
Winter, W E. "Molecular and biochemical analysis of the MODY syndromes." Pediatric diabetes vol. 1,2 (2000): 88-117. doi: https://doi.org/10.1034/j.1399-5448.2000.010206.x
Winter WE. Molecular and biochemical analysis of the MODY syndromes. Pediatr Diabetes. 2000 Jun;1(2):88-117. doi: 10.1034/j.1399-5448.2000.010206.x. PMID: 15016234.
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Pediatr Diabetes. 2000 Jun;1(2):88-117. doi: 10.1034/j.1399-5448.2000.010206.x.

Molecular and biochemical analysis of the MODY syndromes.

Pediatric diabetes

W E Winter

Affiliations

  1. Department of Pathology, Immunology, and Laboratory Medicine, University of Florida, College of Medicine, Box 100275, Gainesville, FL 32610-0275, USA. [email protected]

PMID: 15016234 DOI: 10.1034/j.1399-5448.2000.010206.x

Abstract

Maturity onset diabetes of the young (MODY) is characterized by youth-onset diabetes that is inherited in an autosomal dominant (monogenic) pattern. Classic MODY accounts for less than 5% of cases of childhood diabetes in Caucasians, presents prior to age 25 years, is nonketotic, and may not require insulin treatment. A variant form of MODY that lacks a clearly defined genetic basis occurs in African Americans [atypical diabetes mellitus (ADM)] clinically presents more acutely and is initially insulin requiring. To date, five molecular causes of classic MODY have been identified: hepatocyte nuclear factor-4 alpha (HNF-4 alpha; MODY1), glucokinase (MODY2), hepatocyte nuclear factor-1 alpha (HNF-1 alpha; MODY3), insulin promoter factor-1 (IPF-1, MODY4), and hepatocyte nuclear factor-1 beta (HNF-1 beta; MODY5). MODY is studied as a model of beta cell hypofunction and modest insulinopenia. Clinical recognition of ADM is important for patient management to avoid confusion with type 1 diabetes mellitus.

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