Serum insulin-releasing activity and the Prader-Willi syndrome.
Pathogenesis and etiology of the various clinical features of the Prader-Willi syndrome (PWS) are not completely understood. There is evidence suggesting a hypothalamic abnormality leading to hypogonadism in this syndrome. To test the possible hypothalamic involvement in the pathogenesis of obesity in these patients we studied the sera of 5 patients with PWS for insulin-releasing activity of possible hypothalamic origin. In addition 4 patients with a PWS-like syndrome and 6 obese patients with signs of central nervous system damage were studied. All five patients with PWS showed significant insulin-releasing activity in their sera. Of the four patients with PWS-like symptoms three showed similar activity while three of the obese patients with central nervous system damage had this activity in their sera. Serum insulin-releasing activity has been shown earlier to be associated with childhood obesity and it could play a role in the pathogenesis of obesity in PWS.
D000293:Adolescent; D000328:Adult; D000818:Animals; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007031:Hypothalamus / Q000503:physiopathology; D066298:In Vitro Techniques; D007328:Insulin / Q000378:metabolism*; D000078790:Insulin Secretion; D036341:Intercellular Signaling Peptides and Proteins; D007515:Islets of Langerhans / Q000187:drug effects / Q000378:metabolism; D008297:Male; D009765:Obesity / Q000097:blood; D010455:Peptides / Q000097:blood* / Q000494:pharmacology; D011218:Prader-Willi Syndrome / Q000097:blood*; D051381:Rats
D007328:Insulin; D036341:Intercellular Signaling Peptides and Proteins; D010455:Peptides; C034184:insulin-glucagon liberin