Tryggvason K
Eur J Clin Invest. 1977 Jun;7(3):177-80. doi: 10.1111/j.1365-2362.1977.tb01594.x.
The composition of the glomerular basement membrane (GBM) was studied in three patients with the congenital nephrotic syndrome of the Finnish type (CNF). A decrease was found in the relative amounts of amino acids characteristic of collagen, i.e. 3-hydroxyproline, 4-hydroxyproline, hydroxylysine and glycine. The decrease of hydroxyproline was also apparent in the ratio of 4-hydroxyproline to proline and in direct assays of 4-hydroxyproline in whole glomeruli. The ratio of 3-hydroxyproline to 4-hydroxyproline was decreased by about 40%. Slight increases were found in the amounts of some amino acids. No significant change was found in the glucose content of the GBM whereas the galactose content was slightly decreased. The results suggest a decrease in the relative amount of the collagen component in the GBM of CNF patients, but a decrease in the relative amount of 3-hydroxyproline indicates that other changes also exist.
对三名芬兰型先天性肾病综合征（CNF）患者的肾小球基底膜（GBM）成分进行了研究。发现胶原蛋白特有的氨基酸相对含量有所下降，即3-羟脯氨酸、4-羟脯氨酸、羟赖氨酸和甘氨酸。在4-羟脯氨酸与脯氨酸的比例以及全肾小球中4-羟脯氨酸的直接测定中，羟脯氨酸的减少也很明显。3-羟脯氨酸与4-羟脯氨酸的比例下降了约40%。发现一些氨基酸的含量略有增加。GBM的葡萄糖含量未发现显著变化，而半乳糖含量略有下降。结果表明，CNF患者GBM中胶原蛋白成分的相对含量下降，但3-羟脯氨酸相对含量的下降表明还存在其他变化。
