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Previous Volume 333:1386-1391 November 23, 1995 Number 21 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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Insulin, the central hormone controlling the utilization of fuel, is initially synthesized in pancreatic beta cells as its relatively inactive precursor, proinsulin. A series of enzymes that process prohormones act on proinsulin to produce mature insulin and C peptide. Abnormalities in the secretory products of beta cells are a rare but well-documented cause of impairment in glucose tolerance and hyperinsulinemia.¹

Two specific syndromes have been defined. First, mutations in the insulin gene that lead to the production of biologically ineffective insulin have been described in several families.^2,3,4 All cases described to date have involved heterozygotes who produce normal insulin molecules . . . [Full Text of this Article]

Case History

Methods

Results

Glucose Homeostasis

Processing of Other Prohormones

Investigation of Other Endocrine Systems

            Hypothalamic–Pituitary–Adrenal Function

            Other Endocrine Investigations

Discussion

Source Information

From the Departments of Medicine (S.O., H.G., P.J.H., A.K.) and Clinical Biochemistry (S.O., H.G., P.J.H., A.K., K.T., C.C.), University of Cambridge, Addenbrooke's Hospital, Cambridge, United Kingdom; the Department of Medicine, University of Chicago, Chicago (K.S.P.); and the Department of Medicine, University of Manchester, Hope Hospital, Salford, United Kingdom (A.W., S.G.).

Address reprint requests to Dr. O'Rahilly at the University of Cambridge, Departments of Medicine and Clinical Biochemistry, Addenbrooke's Hospital, Cambridge CB2 2QR, United Kingdom.

References

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