Different intracellular localization of peroxisomal proteins in fibroblasts from patients with aberrant peroxisome assembly.
Different intracellular localization of peroxisomal proteins in fibroblasts from patients with aberrant peroxisome assembly.
复制标题
过氧化物酶体组装异常患者的成纤维细胞中过氧化物酶体蛋白的细胞内定位不同。
DOI：
--
复制
发表时间：
1992
影响因子：
1.5
通讯作者：
T. Hashimoto
中科院分区：
文献类型：
--
作者：
Yasuyuki Suzuki;N. Shimozawa;S. Yajima;T. Orii;S. Yokota;Y. Tashiro;T. Osumi;T. Hashimoto
我们研究了Zellweger综合征和新生儿肾上腺脑白质营养不良患者成纤维细胞中过氧化物酶体蛋白的细胞内定位，其中过氧化物酶体形态缺陷或严重减少。间接免疫荧光染色显示，过氧化氢酶主要是在这些患者的成纤维细胞的胞质溶胶中检测到，但少量的过氧化氢酶被检测到的颗粒图案中的一小部分细胞。双重免疫荧光染色显示，在这些患者中的过氧化氢酶的颗粒也含有酰基辅酶A氧化酶和非特异性脂质转移蛋白。然而，一个70 kD的膜蛋白和3-酮脂酰CoA硫解酶检测在所有细胞中的颗粒图案。细胞标记后使用毛地黄皂苷的亚细胞分级显示，在颗粒部分中检测到少量的酰基辅酶A氧化酶和约一半的硫解酶的前体形式。这些数据表明，过氧化氢酶，酰基辅酶A氧化酶和非特异性脂质转移蛋白的运输和加工的机制是不同的70 kD的膜整合蛋白和3-酮脂酰辅酶A硫解酶。
We investigated intracellular localization of peroxisomal proteins in fibroblasts from patients with Zellweger syndrome and neonatal adrenoleukodystrophy in whom peroxisomes were morphologically deficient or severely decreased. Indirect immunofluorescence staining revealed that catalase was mainly detected in the cytosol of fibroblasts from these patients, but a small amount of catalase was detected in granular pattern in a small percentage of cells. Double immunofluorescence staining revealed that catalase-containing particles in these patients also contained acyl-CoA oxidase and nonspecific lipid transfer protein. However, a 70 kD integral membrane protein and 3-ketoacyl-CoA thiolase were detected in all cells in granular pattern. Subcellular fractionation using digitonin after cell labeling revealed that a small amount of acyl-CoA oxidase and about half of thiolase in the precursor form were detected in the particulate fraction. These data suggest that the mechanisms of the transport and processing of catalase, acyl-CoA oxidase and nonspecific lipid transfer protein are different from those of the 70 kD integral membrane protein and 3-ketoacyl-CoA thiolase.
新生儿发病和 X 连锁肾上腺脑白质营养不良中的过氧化物酶体缺陷。
DOI：
10.1126/science.3964959
发表时间：
1985
期刊：
Science (New York, N.Y.)
影响因子：
--
作者：
Goldfischer,S;Collins,J;Rapin,I;Coltoff-Schiller,B;Chang,CH;Nigro,M;Black,VH;Javitt,NB;Moser,HW;Lazarow,PB
通讯作者：
Lazarow,PB
肾上腺脑白质营养不良中的过氧化物酶体β-氧化酶蛋白：X连锁肾上腺脑白质营养不良和新生儿肾上腺脑白质营养不良之间的区别。
DOI：
10.1073/pnas.84.5.1425
发表时间：
1987
影响因子：
11.1
作者：
Chen,WW;Watkins,PA;Osumi,T;Hashimoto,T;Moser,HW
通讯作者：
Moser,HW