Dominant inheritance of large molecular weight immunoreactive glucagon.
Plasma from some individuals contains substances which are reactive with glucagon antiserum, are larger than 3,500-dalton glucagon, and have been proposed as possible precursors of glucagon. We have evaluated three generations of a kindred in which 9 of 15 members evaluated had elevated plasma levels of large molecular weight immunoreactive glucagon (L-IRG) with an average concentration of 822 pg/ml. The distribution of individuals with elevated L-IRG levels in this pedigree is consistent with autosomal dominant inheritance. Gel filtration of plasma revealed that all affected family members had excessive amounts of two L-IRG peaks, one with a molecular weight of approximately 9,000 daltons and another in the 10,000 to 20,000-dalton range. Oral glucose tolerance tests were nondiabetic and elicited a fall in L-IRG levels, whereas L-IRG concentrations rose dramatically during the infusion of arginine. These L-IRG species may be precursors of 3,500-DALTON GLUCAGON AND MAY BE ELEVATED in this kindred because of an inherited defect in either their synthesis or degradation.
D000293:Adolescent; D000328:Adult; D000368:Aged; D001120:Arginine / Q000494:pharmacology; D002648:Child; D002850:Chromatography, Gel; D005260:Female; D005799:Genes, Dominant; D005934:Glucagon / Q000097:blood / Q000235:genetics* / Q000276:immunology; D005951:Glucose Tolerance Test; D006801:Humans; D008297:Male; D008875:Middle Aged; D008970:Molecular Weight; D010375:Pedigree
D005934:Glucagon; D001120:Arginine