Genomes and Genes
Gene Symbol: NDUFS6
Description: NADH:ubiquinone oxidoreductase subunit S6
Alias: CI-13kA, CI-13kD-A, CI13KDA, NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial, NADH dehydrogenase (ubiquinone) Fe-S protein 6, 13kDa (NADH-coenzyme Q reductase), NADH-ubiquinone oxidoreductase 13 kDa-A subunit, NADH:ubiquinone oxidoreductase NDUFS6 subunit, complex I 13kDa subunit A, complex I, mitochondrial respiratory chain, 13-kD subunit
- CD147 interacts with NDUFS6 in regulating mitochondrial complex I activity and the mitochondrial apoptotic pathway in human malignant melanoma cellsZ Luo
X Chen Department of Dermatology, Xiangya Hospital, Central South University, 87 Xiangya Road, Changsha, Hunan 410008, China
Curr Mol Med 14:1252-64. 2014..The yeast two-hybrid (Y2H) assay identified NDUFS6 (which encodes a subunit of mitochondrial respiratory chain complex I) as a candidate that interacts with CD147 ..
- Accessory NUMM (NDUFS6) subunit harbors a Zn-binding site and is essential for biogenesis of mitochondrial complex IKatarzyna Kmita
Structural Bioenergetics Group, Institute of Biochemistry II, Medical School, Goethe University, 60438 Frankfurt am Main, Germany
Proc Natl Acad Sci U S A 112:5685-90. 2015..We show that the accessory NUMM subunit of complex I (human NDUFS6) harbors a Zn-binding site and resolve its position by X-ray crystallography...
- Proteome Analysis of Renoprotection Mediated by a Novel Cyclic Helix B Peptide in Acute Kidney InjuryCheng Yang
Department of Urology, Zhongshan Hospital, Fudan University, Shanghai, 200032, China
Sci Rep 5:18045. 2015..DEPs in the oxidative phosphorylation pathway elicited by CHBP are NADH-ubiquinone oxidoreductase Fe-S protein 6 (NDUFS6), alpha-aminoadipic semialdehyde synthase (AASS) and ATP-binding cassette sub-family D member 3 (ABCD3)...
- The origin of the supernumerary subunits and assembly factors of complex I: A treasure trove of pathway evolutionDei M Elurbe
Centre for Molecular and Biomolecular Informatics, Radboud Institute for Molecular Life Sciences, Radboud University Medical Center, Nijmegen, The Netherlands Nijmegen Centre for Mitochondrial Disorders, Radboud University Medical Center, Nijmegen, The Netherlands
Biochim Biophys Acta 1857:971-9. 2016..case in which a complex I protein itself appears to have been the source of a new protein from another complex: NDUFS6 gave rise to cytochrome c oxidase subunit COX4/COX5b...
- Tissue-specific splicing of an Ndufs6 gene-trap insertion generates a mitochondrial complex I deficiency-specific cardiomyopathyBi xia Ke
Murdoch Childrens Research Institute, Royal Children s Hospital, Parkville, VIC 3052, Australia
Proc Natl Acad Sci U S A 109:6165-70. 2012..In humans, mutations in the NDUFS6 gene, encoding a CI subunit, cause severe CI deficiency and neonatal death...
- Deficiency in mitochondrial complex I activity due to Ndufs6 gene trap insertion induces renal diseaseJosephine M Forbes
Glycation, Nutrition and Metabolism Laboratory, Baker IDI Heart and Diabetes Institute, Melbourne, Australia
Antioxid Redox Signal 19:331-43. 2013..To determine whether an abnormality in mitochondrial complex I per se is associated with development of renal disease, mice with a knockdown of the complex I gene, Ndufs6 were studied.
- Novel insights into the role of Neurospora crassa NDUFAF2, an evolutionarily conserved mitochondrial complex I assembly factorBruno Pereira
Instituto de Biologia Molecular e Celular, Universidade do Porto, Porto, Portugal
Mol Cell Biol 33:2623-34. 2013..4 (NDUFA12), 18.4 (NDUFS6), and 21 (NDUFS4) kDa. Our results demonstrate that the 13...
- cDNA sequence and chromosomal localization of the remaining three human nuclear encoded iron sulphur protein (IP) subunits of complex I: the human IP fraction is completedJ Loeffen
Nijmegen Center for Mitochondrial Disorders, University Children s Hospital Nijmegen, The Netherlands
Biochem Biophys Res Commun 247:751-8. 1998..human NADH:ubiquinone oxidoreductase subunits of this IP fraction: the NDUFS2 (49 kDa), NDUFS3 (30 kDa), and NDUFS6 (13 kDa) subunits...
- Intron based radiation hybrid mapping of 15 complex I genes of the human electron transport chainT Emahazion
Medical Genetics Unit, Department of Genetics and Pathology, Biomedical Center, Uppsala University, Uppsala, Sweden
Cytogenet Cell Genet 82:115-9. 1998..Radiation hybrid mapping of these PCRs located 15 complex I genes to chromosomes l, 4, 5 (2 genes), 7 (2 genes), 8, 9 (2 genes), 11, 14, 16 (2 genes), 18, and 19...
- NDUFS6 mutations are a novel cause of lethal neonatal mitochondrial complex I deficiencyDenise M Kirby
Murdoch Childrens Research Institute, Royal Children s Hospital, Melbourne, Victoria, Australia
J Clin Invest 114:837-45. 2004..b>NDUFS6, a complex I subunit gene not previously associated with complex I deficiency, was grossly underexpressed in the 2 ..
- Leigh syndrome associated with mitochondrial complex I deficiency due to a novel mutation in the NDUFS1 geneMiguel A Martin
Centro de Investigacion, Hospital Universitario 12 de Octubre, Madrid, Spain
Arch Neurol 62:659-61. 2005..Mutations in the nuclear-encoded subunits of complex I of the mitochondrial respiratory chain are a recognized cause of Leigh syndrome (LS). Recently, 6 mutations in the NDUFS1 gene were identified in 3 families...
- Analysis of the assembly profiles for mitochondrial- and nuclear-DNA-encoded subunits into complex IMichael Lazarou
Department of Biochemistry, La Trobe University, Melbourne, Australia
Mol Cell Biol 27:4228-37. 2007..Subunit exchange may also act as an efficient mechanism to prevent the accumulation of oxidatively damaged subunits that would otherwise be detrimental to mitochondrial oxidative phosphorylation and have the potential to cause disease...
- Integrative genomics analysis of chromosome 5p gain in cervical cancer reveals target over-expressed genes, including DroshaLuigi Scotto
Department of Pathology, Columbia University Medical Center, New York, New York, USA
Mol Cancer 7:58. 2008..These changes may possess oncogenic properties by deregulating tumor-related genes. Gain of short arm of chromosome 5 (5p) is the most frequent karyotypic change in CC...
- Mutated NDUFS6 is the cause of fatal neonatal lactic acidemia in Caucasus JewsRonen Spiegel
Genetic Institute, Ha Emek Medical Center, Afula, Rappaport School of Medicine, Technion, Haifa, Israel
Eur J Hum Genet 17:1200-3. 2009..6 Mb region of identical haplotype in the affected babies. Sequence analysis of the nuclear gene encoding for the NDUFS6 mitochondrial complex I subunit located within this region identified the c...