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Decreased activity of the mitochondrial ATP-synthase in fibroblasts from children with late-infantile and juvenile neuronal ceroid lipofuscinosis.

作者信息

Das A M, Kohlschütter A

机构信息

Department of Pediatrics, Hannover Medical School, Germany.

出版信息

J Inherit Metab Dis. 1996;19(2):130-2. doi: 10.1007/BF01799411.

DOI:10.1007/BF01799411
PMID:8739947
Abstract
摘要

相似文献

1
Decreased activity of the mitochondrial ATP-synthase in fibroblasts from children with late-infantile and juvenile neuronal ceroid lipofuscinosis.
J Inherit Metab Dis. 1996;19(2):130-2. doi: 10.1007/BF01799411.
2
Anomalies of mitochondrial ATP synthase regulation in four different types of neuronal ceroid lipofuscinosis.四种不同类型神经元蜡样脂褐质沉积症中线粒体ATP合酶调节异常。
Mol Genet Metab. 1999 Apr;66(4):349-55. doi: 10.1006/mgme.1999.2811.
3
Increased urine concentration of subunit c of mitochondrial ATP synthase in neuronal ceroid lipofuscinoses patients.神经元蜡样脂褐质沉积症患者线粒体ATP合酶亚基c的尿液浓度升高。
J Inherit Metab Dis. 1994;17(2):205-10. doi: 10.1007/BF00711619.
4
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.
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 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.
7
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.
8
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.
9
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.
10
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.

引用本文的文献

1
Loss of CLN3, the gene mutated in juvenile neuronal ceroid lipofuscinosis, leads to metabolic impairment and autophagy induction in retinal pigment epithelium.CLN3 基因的缺失是少年神经元蜡样脂褐质沉积症的致病基因，会导致视网膜色素上皮的代谢损伤和自噬诱导。
Biochim Biophys Acta Mol Basis Dis. 2020 Oct 1;1866(10):165883. doi: 10.1016/j.bbadis.2020.165883. Epub 2020 Jun 25.
2
Vision loss in juvenile neuronal ceroid lipofuscinosis (CLN3 disease).青少年神经元蜡样脂褐质沉积症（CLN3病）中的视力丧失。
Ann N Y Acad Sci. 2016 May;1371(1):55-67. doi: 10.1111/nyas.12990. Epub 2016 Jan 8.
3
Polyunsaturated fatty acids reverse the lysosomal storage and accumulation of subunit 9 of mitochondrial F1F0-ATP synthase in cultured lymphoblasts from patients with Batten disease.

本文引用的文献

1
Human forms of neuronal ceroid-lipofuscinosis (Batten disease): consensus on diagnostic criteria, Hamburg 1992.人类神经元蜡样脂褐质沉积症（巴滕病）：诊断标准共识，汉堡，1992年
J Inherit Metab Dis. 1993;16(2):241-4. doi: 10.1007/BF00710254.
2
Regulation of the mitochondrial ATP-synthase in human fibroblasts.人成纤维细胞中线粒体ATP合酶的调控
Clin Chim Acta. 1994 Nov;231(1):61-8. doi: 10.1016/0009-8981(94)90254-2.
3
Abnormal degradative pathway of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid-lipofuscinosis (Batten disease).
多不饱和脂肪酸可逆转巴顿病患者培养的成淋巴细胞中线粒体F1F0-ATP合酶亚基9的溶酶体储存和积累。
J Inherit Metab Dis. 1997 Jul;20(3):457-60. doi: 10.1023/a:1005387608456.
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.
4
Human mitochondrial electron transport chain: assay of succinate: cytochrome c reductase in leukocytes, platelets and cultured fibroblasts.人类线粒体电子传递链：白细胞、血小板和培养的成纤维细胞中琥珀酸：细胞色素c还原酶的测定
Biochem Med. 1981 Apr;25(2):234-8. doi: 10.1016/0006-2944(81)90080-6.
5
The binding and release of the inhibitor protein are governed independently by ATP and membrane potential in ox-heart submitochondrial vesicles.在牛心亚线粒体小泡中，抑制蛋白的结合与释放分别由ATP和膜电位独立调控。
Biochim Biophys Acta. 1988 Mar 30;933(1):12-21. doi: 10.1016/0005-2728(88)90051-5.
6
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.