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GeneQ18029898· pop 5· linked from 7 articles

Also known as B8, CD14, CIB8, NADH:ubiquinone oxidoreductase subunit A2, MC1DN13

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 is a protein that in humans is encoded by the NDUFA2 gene. The NDUFA2 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in the NDUFA2 gene are associated with Leigh's syndrome.

Gene data

NDUFA2
Name
NADH:ubiquinone oxidoreductase subunit A2
Type
protein-coding
Aliases
B8, CIB8, MC1DN13

The encoded protein is a subunit of the hydrophobic protein fraction of the NADH:ubiquinone oxidoreductase (complex 1), the first enzyme complex in the electron transport chain located in the inner mitochondrial membrane, and may be involved in regulating complex I activity or its assembly via assistance in redox processes. Mutations in this gene are associated with Leigh syndrome, an early-onset progressive neurodegenerative disorder. Alternative splicing results in multiple transcript variants.[provided by RefSeq, May 2010].

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Wikidata facts

Image
Protein NDUFA2 PDB 1s3a.png
Show 5 more facts
HomoloGene ID
37628
genomic end
140647771
genomic start
140018325
cytogenetic location
5q31.3
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Article

6 sections
Contents
  • Structure
  • Function
  • Clinical significance
  • Interactions
  • References
  • Further reading

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 is a protein that in humans is encoded by the NDUFA2 gene. The NDUFA2 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. Mutations in the NDUFA2 gene are associated with Leigh's syndrome.

== Structure ==

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