Impaired cortisol secretion in abetalipoproteinemia

D. R. Illingworth, Eric Orwoll, W. E. Conner

Research output: Contribution to journalArticle

27 Citations (Scopus)

Abstract

In the adrenal gland cholesterol for steroid biosynthesis is derived from both de novo biosynthesis and receptor mediated uptake of plasma low density lipoproteins (LDL). In the present study we have compared ACTH stimulated adrenal production of cortisol in four control subjects and one adult male patient with abetalipoproteinemia, a disorder in which LDL is absent. Basal morning cortisol levels in the plasma in the control subjects (13.3 ± 1.6 μg/dl) and abetalipoproteinemic patient (14.6 μg/dl) were similar. During infusion of α1, 24 ACTH however, plasma cortisol levels were higher in the control subjects than in the abetalipoproteinemic patient and this difference was significant at times after 4 hours. Urinary excretion of both 17-hydroxy and 17-ketosteroids over the 24 hour infusion period was also significantly lower in the abetalipoproteinemic patient indicating that cortisol production rates were reduced. Our results suggest that in the absence of plasma low density lipoproteins, as occurs in abetalipoproteinemia, the maximal production of adrenal corticosteroids is impaired. By inference, these findings lend in vivo support to the view that plasma low density lipoproteins serve as an important source of cholesterol for adrenal steroidogenesis in man.

Original languageEnglish (US)
Pages (from-to)977-979
Number of pages3
JournalJournal of Clinical Endocrinology and Metabolism
Volume50
Issue number5
StatePublished - 1980

Fingerprint

Abetalipoproteinemia
Hydrocortisone
LDL Lipoproteins
Plasmas
Biosynthesis
Cholesterol
Cosyntropin
17-Ketosteroids
Adrenal Glands
Adrenocorticotropic Hormone
Adrenal Cortex Hormones
Steroids

ASJC Scopus subject areas

  • Biochemistry
  • Endocrinology, Diabetes and Metabolism

Cite this

Impaired cortisol secretion in abetalipoproteinemia. / Illingworth, D. R.; Orwoll, Eric; Conner, W. E.

In: Journal of Clinical Endocrinology and Metabolism, Vol. 50, No. 5, 1980, p. 977-979.

Research output: Contribution to journalArticle

Illingworth, D. R. ; Orwoll, Eric ; Conner, W. E. / Impaired cortisol secretion in abetalipoproteinemia. In: Journal of Clinical Endocrinology and Metabolism. 1980 ; Vol. 50, No. 5. pp. 977-979.
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