Ensinck J W, Palmer J P
Metabolism. 1976 Nov;25(11 Suppl 1):1409-11. doi: 10.1016/s0026-0495(76)80153-9.
Excessive plasma levels of high molecular weight (MW) components of immunoreactive glucagon (IRG) have been found in three generations of a family in a pattern consistent with an autosomal dominant trait. Preliminary studies using gel filtration suggest the presence of two MW species of IRG greater than 3500 daltons in plasma of affected members who have normal oral glucose tolerance tests. The "hyperglucagonemia" in this pedigree is consonant with either a structural defect in biosynthetic precursors or an abnormality in enzyme-mediated cleavage of proglucagon.
在一个家族的三代人中发现,免疫反应性胰高血糖素(IRG)的高分子量(MW)成分血浆水平过高,其模式符合常染色体显性性状。使用凝胶过滤的初步研究表明,在口服葡萄糖耐量试验正常的受影响成员的血浆中存在两种分子量大于3500道尔顿的IRG。该谱系中的“高胰高血糖素血症”与生物合成前体的结构缺陷或胰高血糖素原的酶介导裂解异常相一致。