Ono S, Mechanic G L, Yamauchi M
Dental Research Center, University of North Carolina, Chapel Hill 27599-7455.
J Neurol Sci. 1990 Dec;100(1-2):234-7. doi: 10.1016/0022-510x(90)90038-o.
The skin tissues from patients with amyotrophic lateral sclerosis (ALS) and controls were studied by electron microscopy, and their amino acid compositions were examined. On electron microscopy, the most conspicuous findings in ALS were (1) a marked increase in amorphous material separating collagen bundles, and (2) the smaller diameter of collagen fibrils. These were more marked with longer duration of illness. Amino acid analysis showed that the levels of hydroxyproline, hydroxylysine, and glycine were significantly low (P less than 0.001, P less than 0.01, and P less than 0.001, respectively) in ALS patients as compared with those of controls, and there was a significant negative correlation between the level of hydroxyproline and duration of illness in ALS patients (r = -0.88, P less than 0.01). In addition, the collagen content per dry weight (mg) of the tissues in ALS was significantly smaller (P less than 0.001) than in controls. These results indicate that the metabolism of skin collagen might be affected in the disease process of ALS.
对肌萎缩侧索硬化症（ALS）患者和对照组的皮肤组织进行了电子显微镜研究，并检测了它们的氨基酸组成。在电子显微镜下，ALS最显著的发现是：（1）分隔胶原束的无定形物质显著增加；（2）胶原纤维直径变小。病程越长，这些变化越明显。氨基酸分析表明，与对照组相比，ALS患者的羟脯氨酸、羟赖氨酸和甘氨酸水平显著降低（分别为P<0.001、P<0.01和P<0.001），并且ALS患者的羟脯氨酸水平与病程之间存在显著负相关（r = -0.88，P<0.01）。此外，ALS患者组织每干重（mg）的胶原蛋白含量显著低于对照组（P<0.001）。这些结果表明，在ALS的疾病过程中，皮肤胶原蛋白的代谢可能受到影响。
