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Uridine preserves the expression of respiratory enzyme deficiencies in cultured fibroblasts.

作者信息

Gérard B, Bourgeron T, Chretien D, Rötig A, Munnich A, Rustin P

出版信息

Eur J Pediatr. 1993 Mar;152(3):270. doi: 10.1007/BF01956163.

DOI:10.1007/BF01956163
PMID:8383055
Abstract
摘要

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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]
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Evolutionarily conserved susceptibility of the mitochondrial respiratory chain to SDHI pesticides and its consequence on the impact of SDHIs on human cultured cells.线粒体呼吸链对 SDHI 类农药的进化保守易感性及其对 SDHIs 类农药对人培养细胞影响的后果。
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Profiling the mitochondrial proteome of Leber's Hereditary Optic Neuropathy (LHON) in Thailand: down-regulation of bioenergetics and mitochondrial protein quality control pathways in fibroblasts with the 11778G>A mutation.

本文引用的文献

1
Chick embryo cells rendered respiration-deficient by chloramphenicol and ethidium bromide are auxotrophic for pyrimidines.用氯霉素和溴化乙锭处理后导致呼吸缺陷的鸡胚细胞对嘧啶呈营养缺陷型。
Biochem Biophys Res Commun. 1980 May 14;94(1):71-7. doi: 10.1016/s0006-291x(80)80189-6.
2
Human cells lacking mtDNA: repopulation with exogenous mitochondria by complementation.缺乏线粒体DNA的人类细胞：通过互补作用由外源性线粒体进行重新填充。
Science. 1989 Oct 27;246(4929):500-3. doi: 10.1126/science.2814477.
3
The use of skin fibroblast cultures in the detection of respiratory chain defects in patients with lacticacidemia.
泰国莱伯遗传性视神经病变（LHON）线粒体蛋白质组分析：11778G>A突变的成纤维细胞中生物能量学和线粒体蛋白质质量控制途径的下调
PLoS One. 2014 Sep 12;9(9):e106779. doi: 10.1371/journal.pone.0106779. eCollection 2014.
4
Mitochondrial biochemical activities and heteroplasmy evolution in established D. subobscura cell line.
In Vitro Cell Dev Biol Anim. 2006 Jul-Aug;42(7):201-7. doi: 10.1290/0601003.1.
5
Mitochondrial disorders: prevalence, myths and advances.线粒体疾病：患病率、误解与进展
J Inherit Metab Dis. 2004;27(3):349-62. doi: 10.1023/B:BOLI.0000031098.41409.55.
6
Laboratory approach to mitochondrial diseases.线粒体疾病的实验室检测方法
J Physiol Biochem. 2001 Sep;57(3):267-84. doi: 10.1007/BF03179820.
7
Altered mitochondrial function in fibroblasts containing MELAS or MERRF mitochondrial DNA mutations.含有线粒体脑肌病伴乳酸血症和卒中样发作（MELAS）或肌阵挛性癫痫伴破碎红纤维综合征（MERRF）线粒体DNA突变的成纤维细胞中线粒体功能的改变
Biochem J. 1996 Sep 1;318 ( Pt 2)(Pt 2):401-7. doi: 10.1042/bj3180401.
8
Clinical presentations and laboratory investigations in respiratory chain deficiency.呼吸链缺陷的临床表现及实验室检查
Eur J Pediatr. 1996 Apr;155(4):262-74. doi: 10.1007/BF02002711.
9
A mitochondrial DNA microdeletion in a newborn girl with transient lactic acidosis.
J Inherit Metab Dis. 1996;19(2):115-8. doi: 10.1007/BF01799407.
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.
4
Prenatal diagnosis of cytochrome c oxidase deficiency in cultured amniocytes is hazardous.
Prenat Diagn. 1992 Jun;12(6):548-9. doi: 10.1002/pd.1970120614.