Studies of fat mobilization and transport are reported in six patients with the Prader-Willi syndrome. Two patients had carbohydrate intolerance. One of these had a low and the other an augmented insulin response to glucose challenge. Following challenge with glucose, three of the four nondiabetics had normal insulin responses or increased responses consistent with their obesity; the other nondiabetic had insulinopenia. Measurements of the effects of norepinephrine, insulin, glucose, and 5-methylpyrazole-3-carboxylic acid on plasma levels of FFA, glycerol, and ketones provide no evidence for abnormal regulation of mobilization of fat from adipose tissue. Measurements of plasma lipids and postheparin lipolytic activity are consistent with normal uptake of fat into adipose tissue, and normal fatty acid composition of adipose tissue gives no evidence for abnormal Upogenesis.
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Original Contributions|
September 01 1977
The Prader-Willi Syndrome Regulation of Fat Transport
D M Bier, MD;
D M Bier, MD
Cardiovascular Research Institute and the Departments of Pediatrics and Medicine, University of California School of Medicine
San Francisco, California 94143
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S L Kaplan, MD;
S L Kaplan, MD
Cardiovascular Research Institute and the Departments of Pediatrics and Medicine, University of California School of Medicine
San Francisco, California 94143
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R J Havel, MD
R J Havel, MD
Cardiovascular Research Institute and the Departments of Pediatrics and Medicine, University of California School of Medicine
San Francisco, California 94143
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Address reprint requests to D. M. Bier, M. D., Washington University School of Medicine, Department of Medicine- Metabolism, 660 S. Euclid, St. Louis, Missouri 63110.
1
Dr. Bier was a Bay Area Heart Association Junior Fellow. Present address: Division of Endocrinology and Metabolism, Washington University School of Medicine, St. Louis, Missouri 63110.
2
Dr. Kaplan was the recipient of a Research Career Development Award, National Institute of Child Health and Human Development, National Institutes of Health, K3-HD-8702.
Citation
D M Bier, S L Kaplan, R J Havel; The Prader-Willi Syndrome Regulation of Fat Transport. Diabetes 1 September 1977; 26 (9): 874–881. https://doi.org/10.2337/diab.26.9.874
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