Endothelin‐1 in the brain of patients with galactosialidosis: Its abnormal increase and distribution pattern
Endothelin‐1 in the brain of patients with galactosialidosis: Its abnormal increase and distribution pattern
复制标题
半乳糖唾液酸贮积症患者脑内内皮素-1的异常升高和分布模式
作者：
K. Itoh;K. Oyanagi;H. Takahashi;Takeshi Sato;Y. Hashizume;M. Shimmoto;H. Sakuraba
内皮素-1是具有丝氨酸羧肽酶活性的溶酶体保护蛋白/组织蛋白酶A（PPCA）的体外肽底物。已证实，在患有半乳糖唾液酸沉积症（一种人类PPCA缺乏症）的患者的尸检脑区（包括小脑、海马结构和脊髓）内，内皮素-1特异性免疫反应性显著增加，并在神经元和神经胶质细胞中分布异常。PPCA的内皮素-1降解活性的遗传缺陷被认为是导致这种疾病的一些神经异常的原因。《神经病学年鉴》2000; 47：122-126
Endothelin‐1 is a peptidic substrate in vitro of lysosomal protective protein/cathepsin A (PPCA) with serine carboxypeptidase activity. Endothelin‐1–specific immunoreactivity has been demonstrated to be markedly increased and distributed abnormally in the neurons and glial cells within autopsied brain regions, including the cerebellum, hippocampal formation, and spinal cord, of patients affected with galactosialidosis, a human PPCA deficiency. The genetic defect of the endothelin‐1 degrading activity of PPCA is suggested to cause some of the neurological abnormalities of this disease. Ann Neurol 2000; 47:122–126
强效血管收缩剂内皮素在人类中枢神经系统中的表达。
DOI：
10.1172/jci114677
发表时间：
1990
期刊：
The Journal of clinical investigation
影响因子：
--
作者：
Lee,ME;delaMonte,SM;Ng,SC;Bloch,KD;Quertermous,T
通讯作者：
Quertermous,T
DOI：
10.1161/01.hyp.21.6.925
发表时间：
1993
期刊：
Hypertension (Dallas, Tex. : 1979)
影响因子：
--
作者：
Jackman,HL;Morris,PW;Rabito,SF;Johansson,GB;Skidgel,RA;Erdös,EG
通讯作者：
Erdös,EG