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NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 5, 13kDa
Symbols NDUFA5 ; B13; CI-13KD-B; CI-13kB; NUFM; UQOR13
External IDs OMIM601677 MGI1915452 HomoloGene3664 GeneCards: NDUFA5 Gene
EC number,
RNA expression pattern
PBB GE NDUFA5 201304 at tn.png
PBB GE NDUFA5 215850 s at tn.png
More reference expression data
Species Human Mouse
Entrez 4698 68202
Ensembl ENSG00000128609 ENSMUSG00000023089
UniProt Q16718 Q9CPP6
RefSeq (mRNA) NM_005000 NM_026614
RefSeq (protein) NP_004991 NP_080890
Location (UCSC) Chr 7:
123.18 – 123.2 Mb
Chr 6:
24.52 – 24.53 Mb
PubMed search [1] [2]

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 is an enzyme that in humans is encoded by the NDUFA5 gene.[1][2][3]

The human NDUFA5 gene codes for the B13 subunit of complex I of the respiratory chain, which transfers electrons from NADH to ubiquinone. The high degree of conservation of NDUFA5 extending to plants and fungi indicates its functional significance in the enzyme complex.

The protein localizes to the inner mitochondrial membrane as part of the 7 component-containing, water soluble iron-sulfur protein (IP) fraction of complex I, although its specific role is unknown. It is assumed to undergo post-translational removal of the initiator methionine and N-acetylation of the next amino acid. The predicted secondary structure is primarily alpha helix, but the carboxy-terminal half of the protein has high potential to adopt a coiled-coil form. The amino-terminal part contains a putative beta sheet rich in hydrophobic amino acids that may serve as mitochondrial import signal. Related pseudogenes have also been identified on four other chromosomes.[3]


  1. ^ Emahazion T, Beskow A, Gyllensten U, Brookes AJ (Nov 1998). "Intron based radiation hybrid mapping of 15 complex I genes of the human electron transport chain". Cytogenet Cell Genet 82 (1-2): 115–9. doi:10.1159/000015082. PMID 9763677. 
  2. ^ Russell MW, du Manoir S, Collins FS, Brody LC (Mar 1997). "Cloning of the human NADH: ubiquinone oxidoreductase subunit B13: localization to chromosome 7q32 and identification of a pseudogene on 11p15". Mamm Genome 8 (1): 60–1. doi:10.1007/s003359900350. PMID 9021153. 
  3. ^ a b "Entrez Gene: NDUFA5 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 5, 13kDa". 

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