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Detection of respiratory chain dysfunction by measuring lactate and pyruvate production in cultured fibroblasts.

作者信息

Wijburg F A, Feller N, Ruitenbeek W, Trijbels J M, Sengers R C, Scholte H R, Przyrembel H, Wanders R J

机构信息

Department of Pediatrics, University Hospital of Amsterdam (AMC), The Netherlands.

出版信息

J Inherit Metab Dis. 1990;13(3):355-8. doi: 10.1007/BF01799394.

DOI:10.1007/BF01799394
PMID:2172645
Abstract
摘要

相似文献

1
Detection of respiratory chain dysfunction by measuring lactate and pyruvate production in cultured fibroblasts.通过测量培养的成纤维细胞中乳酸和丙酮酸的生成来检测呼吸链功能障碍。
J Inherit Metab Dis. 1990;13(3):355-8. doi: 10.1007/BF01799394.
2
Studies on the formation of lactate and pyruvate from glucose in cultured skin fibroblasts: implications for detection of respiratory chain defects.培养的皮肤成纤维细胞中葡萄糖生成乳酸和丙酮酸的研究：对呼吸链缺陷检测的意义
Biochem Int. 1989 Sep;19(3):563-70.
3
Prenatal diagnosis of systemic disorders of the respiratory chain in cultured amniocytes and chorionic villus fibroblasts by studying the formation of lactate and pyruvate from glucose.通过研究葡萄糖生成乳酸和丙酮酸的过程，对培养的羊膜细胞和绒毛膜绒毛成纤维细胞中的呼吸链系统疾病进行产前诊断。
J Inherit Metab Dis. 1992;15(1):84-91. doi: 10.1007/BF01800349.
4
Respiratory chain defects in the mitochondria of cultured skin fibroblasts from three patients with lacticacidemia.三名乳酸血症患者培养的皮肤成纤维细胞线粒体中的呼吸链缺陷
J Clin Invest. 1986 May;77(5):1422-7. doi: 10.1172/JCI112453.
5
The use of skin fibroblast cultures in the detection of respiratory chain defects in patients with lacticacidemia.皮肤成纤维细胞培养在检测乳酸血症患者呼吸链缺陷中的应用。
Pediatr Res. 1990 Nov;28(5):549-55. doi: 10.1203/00006450-199011000-00027.
6
Screening for disorders of pyruvate metabolism by measuring the ratio of the rates of lactate production and pyruvate decarboxylation in cultured skin fibroblasts.通过测量培养的皮肤成纤维细胞中乳酸生成速率与丙酮酸脱羧速率的比值来筛查丙酮酸代谢紊乱。
Clin Chim Acta. 1991 May 31;199(1):17-22. doi: 10.1016/0009-8981(91)90004-v.
7
Clinical presentation of mitochondrial respiratory chain defects in NADH-coenzyme Q reductase and cytochrome oxidase: clues to pathogenesis of Leigh disease.NADH辅酶Q还原酶和细胞色素氧化酶中线粒体呼吸链缺陷的临床表现：对Leigh病发病机制的线索
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[Value of skin fibroblasts in culture for the diagnosis of mitochondrial cell dysfunction. Apropos of 5 cases with cytochrome c oxidase deficiency].[培养的皮肤成纤维细胞在诊断线粒体细胞功能障碍中的价值。关于5例细胞色素c氧化酶缺乏症]
Pediatrie. 1993;48(4):287-95.
9
Deficiency of the reduced nicotinamide adenine dinucleotide dehydrogenase component of complex I of mitochondrial electron transport. Fatal infantile lactic acidosis and hypermetabolism with skeletal-cardiac myopathy and encephalopathy.线粒体电子传递复合体I中还原型烟酰胺腺嘌呤二核苷酸脱氢酶成分缺乏。致命性婴儿乳酸酸中毒和伴有骨骼肌-心肌病及脑病的高代谢。
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Menadione partially restores NADH-oxidation and ATP-synthesis in complex I deficient fibroblasts.甲萘醌可部分恢复复合体I缺陷型成纤维细胞中的NADH氧化和ATP合成。
Biochem Int. 1990 Oct;22(2):303-9.

引用本文的文献

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Lactic acidosis in long-chain fatty acid beta-oxidation disorders.长链脂肪酸β氧化障碍中的乳酸性酸中毒
J Inherit Metab Dis. 1998 Aug;21(6):645-54. doi: 10.1023/a:1005480516801.
2
Problems with the biochemical diagnosis in mitochondrial (encephalo-)myopathies.线粒体（脑）肌病的生化诊断问题
Eur J Pediatr. 1993 Mar;152(3):178-84. doi: 10.1007/BF01956139.
3
Respiratory chain function in Leber's hereditary optic neuropathy: lack of correlation with clinical disease.莱伯遗传性视神经病变中的呼吸链功能：与临床疾病缺乏相关性

本文引用的文献

1
Oxidative phosphorylation in mitochondria isolated from human fibroblasts.从人成纤维细胞分离出的线粒体中的氧化磷酸化作用。
Biochim Biophys Acta. 1973 Jan 18;292(1):73-7. doi: 10.1016/0005-2728(73)90251-x.
2
A mitochondrial encephalomyopathy: the first case with an established defect at the level of coenzyme Q.一种线粒体脑肌病：首例辅酶Q水平存在明确缺陷的病例。
Eur J Pediatr. 1986 Feb;144(5):441-4. doi: 10.1007/BF00441735.
3
Clinical presentation of mitochondrial respiratory chain defects in NADH-coenzyme Q reductase and cytochrome oxidase: clues to pathogenesis of Leigh disease.
J Inherit Metab Dis. 1993;16(3):531-3. doi: 10.1007/BF00711673.
4
Morphology of the mitochondria in heat shock protein 60 deficient fibroblasts from mitochondrial myopathy patients. Effects of stress conditions.线粒体肌病患者热休克蛋白60缺陷成纤维细胞中线粒体的形态。应激条件的影响。
Virchows Arch. 1995;427(2):159-65. doi: 10.1007/BF00196521.
5
Restoration of NADH-oxidation in complex I and complex III deficient fibroblasts by menadione.通过甲萘醌恢复复合体I和复合体III缺陷型成纤维细胞中的NADH氧化作用。
J Inherit Metab Dis. 1991;14(3):293-6. doi: 10.1007/BF01811685.
6
Prenatal diagnosis of systemic disorders of the respiratory chain in cultured amniocytes and chorionic villus fibroblasts by studying the formation of lactate and pyruvate from glucose.通过研究葡萄糖生成乳酸和丙酮酸的过程，对培养的羊膜细胞和绒毛膜绒毛成纤维细胞中的呼吸链系统疾病进行产前诊断。
J Inherit Metab Dis. 1992;15(1):84-91. doi: 10.1007/BF01800349.
NADH辅酶Q还原酶和细胞色素氧化酶中线粒体呼吸链缺陷的临床表现：对Leigh病发病机制的线索
J Pediatr. 1987 Feb;110(2):216-22. doi: 10.1016/s0022-3476(87)80157-9.
4
Respiratory chain defects in the mitochondria of cultured skin fibroblasts from three patients with lacticacidemia.三名乳酸血症患者培养的皮肤成纤维细胞线粒体中的呼吸链缺陷
J Clin Invest. 1986 May;77(5):1422-7. doi: 10.1172/JCI112453.
5
Isozymes of cytochrome-c oxidase: characterization and isolation from different tissues.细胞色素 c 氧化酶的同工酶：不同组织中的特性与分离
Methods Enzymol. 1986;126:32-45. doi: 10.1016/s0076-6879(86)26006-1.
6
Defects in oxidative phosphorylation. Biochemical investigations in skeletal muscle and expression of the lesion in other cells.氧化磷酸化缺陷。骨骼肌的生化研究及其他细胞中病变的表现。
J Inherit Metab Dis. 1987;10 Suppl 1:81-97. doi: 10.1007/BF01812849.
7
Studies on the formation of lactate and pyruvate from glucose in cultured skin fibroblasts: implications for detection of respiratory chain defects.培养的皮肤成纤维细胞中葡萄糖生成乳酸和丙酮酸的研究：对呼吸链缺陷检测的意义
Biochem Int. 1989 Sep;19(3):563-70.