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Clinical Consequences of Defects in β-Cell Genes

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Molecular Basis of Pancreas Development and Function

Part of the book series: Endocrine Updates ((ENDO,volume 11))

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Abstract

Genes critical for β-cell function have been identified by the genetic analysis of families with maturity onset diabetes of the young (MODY). The clinical description of MODY families by Fajans and Tattersall (1,2) and the development of appropriate molecular genetic techniques were both critical in the definition of these genes. In this chapter, we describe the clinical and physiological features of patients with known defects in β-cell genes. This has led to the demonstration of previously un-recognized heterogeneity in clinically defined MODY. In addition, it has given fascinating insights into the role of the genes in the normal β-cell both in post-natal and foetal life.

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Authors and Affiliations

  1. Department of Vascular Medicine and Diabetes Research School of Postgraduate Medicine and Health Sciences Barrack Road, Exeter, Devon, EX2 SAX, UK

    Julie C. Evans B.Sc., Timothy M. Frayling Ph.D. & Andrew T. Hattersley D.M.

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  1. Julie C. Evans B.Sc.
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  2. Timothy M. Frayling Ph.D.
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  3. Andrew T. Hattersley D.M.
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Editors and Affiliations

  1. Laboratory of Molecular Endocrinology, Howard Hughes Medical Institute, Massachusetts General Hospital, Boston, MA

    Joel F. Habener M.D.

  2. Department of Medicine, Clinical and Molecular Endocrinology, New York University Medical Center, New York, NY

    Mehboob A. Hussain M.D.

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Evans, J.C., Frayling, T.M., Hattersley, A.T. (2001). Clinical Consequences of Defects in β-Cell Genes. In: Habener, J.F., Hussain, M.A. (eds) Molecular Basis of Pancreas Development and Function. Endocrine Updates, vol 11. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-1669-9_19

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