Cited by 237Pancreatic function and diabetesDiabetes and associated disordersAdrenal Hormones and Disorders

Impaired Processing of Prohormones Associated with Abnormalities of Glucose Homeostasis and Adrenal Function

Open access
O'Rahilly, Stephen, Gray, Helen, Humphreys, Phillippa J., Krook, Anna, Polonsky, Kenneth S., White, Anne, Gibson, Sarah, Taylor, Kevin +1 more·1995·New England Journal of Medicine

Abstract

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.2–4 All cases described to date have involved heterozygotes who produce normal insulin . . .

Integrity signals

Registry

Crossref · 10.1056/nejm199511233332104

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32 of 32 references verified0 citation errors · 0 not checkable

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  • The first report of a patient with congenital NEC 1 deficiency associated with obesity and hypogonadotropic hypogonadism was published in 1995.

    “The first report of a patient with congenital NEC 1 deficiency associated with obesity and hypogonadotropic hypogonadism was published in 1995.65”

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Authors
O'Rahilly, S., Gray, H., Humphreys, P. J., Krook, A., Polonsky, K. S., White, A., Gibson, S., Taylor, K., & Carr, C.
Affiliations
  • University of Cambridge (GB)
  • University of Chicago (US)
  • University of Manchester (GB)
Venue
New England Journal of Medicine
Published
1995-11-23
Volume
333
Issue
21
Pages
1386-1391
ISSN
  • 0028-4793 (ISSN-L)
  • 1533-4406
OpenAlex
W2334031918
Cited by
237
References
32
Citations by year
2026: 3 · 2025: 8 · 2024: 3 · 2023: 8 · 2022: 9 · 2021: 13 · 2020: 11 · 2019: 3 · 2018: 9 · 2017: 9 · 2016: 7 · 2015: 7 · 2014: 3 · 2013: 14 · 2012: 4
FWCI
8.83
Type
journal article
Language
en
Open access
Locations
  • New England Journal of Medicine (publishedVersion)
  • PubMed (publishedVersion)
  • Research Explorer (The University of Manchester) (publishedVersion)
Funders
Wellcome Trust
Topics
  • Health Sciences › Medicine › Surgery › Pancreatic function and diabetes
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Diabetes and associated disorders
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
Keywords
proinsulin processing, prohormone processing, insulin gene mutations, glucose tolerance, hyperinsulinemia, C-peptide, pancreatic beta cells, glucose homeostasis, adrenal function
MeSH terms
Adult, Blood Glucose, Female, Homeostasis, Humans, Hydrocortisone, Hypoglycemia, Hypogonadism, Hypothalamo-Hypophyseal System, Insulin, Pituitary-Adrenal System, Proinsulin, Protein Precursors

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