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Molecular basis of lysosomal accumulation of subunit c of mitochondrial ATP synthase in neuronal ceroid-lipofuscinosis.

作者信息

Ezaki J, Wolfe L S, Kominami E

机构信息

Department of Biochemistry, Juntendo University School of Medicine, Tokyo, Japan.

出版信息

J Inherit Metab Dis. 1993;16(2):296-8. doi: 10.1007/BF00710269.

DOI:10.1007/BF00710269
PMID:8411985
Abstract
摘要

相似文献

1
Molecular basis of lysosomal accumulation of subunit c of mitochondrial ATP synthase in neuronal ceroid-lipofuscinosis.神经元蜡样脂褐质沉积症中线粒体ATP合酶亚基c溶酶体蓄积的分子基础
J Inherit Metab Dis. 1993;16(2):296-8. doi: 10.1007/BF00710269.
2
Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis).线粒体ATP合酶亚基c在巴滕病（蜡样脂褐质沉积症）中的溶酶体储存。
Biochem J. 1991 Apr 1;275 ( Pt 1)(Pt 1):269-72. doi: 10.1042/bj2750269.
3
Specific storage of subunit c of mitochondrial ATP synthase in lysosomes of neuronal ceroid lipofuscinosis (Batten's disease).线粒体ATP合酶亚基c在神经元蜡样脂褐质沉积症（巴滕病）溶酶体中的特异性储存。
J Biochem. 1992 Feb;111(2):278-82. doi: 10.1093/oxfordjournals.jbchem.a123749.
4
Tripeptidyl peptidase I, the late infantile neuronal ceroid lipofuscinosis gene product, initiates the lysosomal degradation of subunit c of ATP synthase.三肽基肽酶I，即晚发性婴儿神经元蜡样脂褐质沉积症基因产物，启动ATP合酶亚基c的溶酶体降解。
J Biochem. 2000 Sep;128(3):509-16. doi: 10.1093/oxfordjournals.jbchem.a022781.
5
Lysosomal storage of the DCCD reactive proteolipid subunit of mitochondrial ATP synthase in human and ovine ceroid lipofuscinoses.人及羊蜡样脂褐质沉积症中线粒体ATP合酶的二环己基碳二亚胺反应性蛋白脂质亚基的溶酶体储存。
Adv Exp Med Biol. 1989;266:211-22; discussion 223. doi: 10.1007/978-1-4899-5339-1_15.
6
Specific delay in the degradation of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid lipofuscinosis is derived from cellular proteolytic dysfunction rather than structural alteration of subunit c.晚期婴儿型神经元蜡样脂褐质沉积症中线粒体ATP合酶亚基c降解的特异性延迟源于细胞蛋白水解功能障碍，而非亚基c的结构改变。
J Neurochem. 1996 Oct;67(4):1677-87. doi: 10.1046/j.1471-4159.1996.67041677.x.
7
Specific delay of degradation of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid lipofuscinosis (Batten disease).晚期婴儿型神经元蜡样脂褐质沉积症（巴滕病）中线粒体ATP合酶亚基c降解的特异性延迟
J Neurochem. 1995 Feb;64(2):733-41. doi: 10.1046/j.1471-4159.1995.64020733.x.
8
Abnormal degradative pathway of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid-lipofuscinosis (Batten disease).线粒体ATP合酶亚基c在晚期婴儿神经元蜡样脂褐质沉积症（巴滕病）中的异常降解途径。
Am J Med Genet. 1995 Jun 5;57(2):254-9. doi: 10.1002/ajmg.1320570229.
9
Mitochondrial ATP synthase subunit c storage in the ceroid-lipofuscinoses (Batten disease).线粒体ATP合酶亚基c在蜡样脂褐质沉积症（巴滕病）中的储存情况。
Am J Med Genet. 1992 Feb 15;42(4):561-7. doi: 10.1002/ajmg.1320420428.
10
Lysine methylation of mitochondrial ATP synthase subunit c stored in tissues of dogs with hereditary ceroid lipofuscinosis.遗传性类脂褐质病犬组织中线粒体ATP合酶亚基c的赖氨酸甲基化
J Biol Chem. 1994 Apr 1;269(13):9906-11.

引用本文的文献

1
Batten disease (ceroid-lipofuscinosis): the enigma of subunit c of mitochondrial ATP synthase accumulation.巴滕病（蜡样脂褐质沉积症）：线粒体ATP合酶亚基c蓄积之谜。
Neurochem Res. 1995 Nov;20(11):1301-4. doi: 10.1007/BF00992504.
2
Elevated levels of neutrophil 4-hydroxynonenal in canine neuronal ceroid-lipofuscinosis and human immortalized lymphocytes of NCL patients.
J Inherit Metab Dis. 1993;16(2):323-9. doi: 10.1007/BF00710275.

本文引用的文献

1
Sequestration of cytoplasmic enzymes in an autophagic vacuole-lysosomal system induced by injection of leupeptin.通过注射亮抑酶肽诱导细胞质酶在自噬泡-溶酶体系统中隔离。
J Biol Chem. 1983 May 25;258(10):6093-100.
2
Lysosomal storage of the DCCD reactive proteolipid subunit of mitochondrial ATP synthase in human and ovine ceroid lipofuscinoses.人及羊蜡样脂褐质沉积症中线粒体ATP合酶的二环己基碳二亚胺反应性蛋白脂质亚基的溶酶体储存。
Adv Exp Med Biol. 1989;266:211-22; discussion 223. doi: 10.1007/978-1-4899-5339-1_15.
3
Ovine ceroid lipofuscinosis. The major lipopigment protein and the lipid-binding subunit of mitochondrial ATP synthase have the same NH2-terminal sequence.
绵羊蜡样脂褐质沉积症。主要脂色素蛋白与线粒体ATP合酶的脂质结合亚基具有相同的NH2末端序列。
J Biol Chem. 1989 Apr 5;264(10):5736-40.
4
The sequence of the major protein stored in ovine ceroid lipofuscinosis is identical with that of the dicyclohexylcarbodiimide-reactive proteolipid of mitochondrial ATP synthase.存储于绵羊蜡样脂褐质病中的主要蛋白质序列与线粒体ATP合酶的二环己基碳二亚胺反应性蛋白脂质序列相同。
Biochem J. 1990 Jun 15;268(3):751-8. doi: 10.1042/bj2680751.
5
Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis).线粒体ATP合酶亚基c在巴滕病（蜡样脂褐质沉积症）中的溶酶体储存。
Biochem J. 1991 Apr 1;275 ( Pt 1)(Pt 1):269-72. doi: 10.1042/bj2750269.
6
Infantile form of neuronal ceroid lipofuscinosis (CLN1) maps to the short arm of chromosome 1.
Genomics. 1991 Jan;9(1):170-3. doi: 10.1016/0888-7543(91)90235-7.
7
Specific storage of subunit c of mitochondrial ATP synthase in lysosomes of neuronal ceroid lipofuscinosis (Batten's disease).线粒体ATP合酶亚基c在神经元蜡样脂褐质沉积症（巴滕病）溶酶体中的特异性储存。
J Biochem. 1992 Feb;111(2):278-82. doi: 10.1093/oxfordjournals.jbchem.a123749.