NDUFB11

NDUFB11
Identifiers
Aliases	NDUFB11, CI-ESSS, ESSS, NP17.3, Np15, P17.3, LSDMCA3, NADH:ubiquinone oxidoreductase subunit B11
External IDs	MGI: 1349919 HomoloGene: 10398 GeneCards: NDUFB11
Gene ontology Molecular function • protein binding Cellular component • membrane • respiratory chain • mitochondrial respiratory chain complex I • integral component of membrane • mitochondrion • mitochondrial inner membrane Biological process • oxidation-reduction process • mitochondrial respiratory chain complex I assembly • mitochondrial electron transport, NADH to ubiquinone Sources:Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	54539	104130
Ensembl	ENSG00000147123	ENSMUSG00000031059
UniProt	Q9NX14	O09111
RefSeq (mRNA)	NM_019056 NM_001135998	NM_019435
RefSeq (protein)	NP_001129470.1 NP_061929.2	NP_062308.2
Location (UCSC)	Chr X: 47.14 – 47.15 Mb	Chr X: 20.62 – 20.62 Mb
PubMed search	[1]	[2]
Wikidata

NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial (NADH-ubiquinone oxidoreductase ESSS subunit) is an enzyme that in humans is encoded by the NDUFB11 gene.^[3][4][5] NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 11 is an accessory subunit of the NADH dehydrogenase (ubiquinone) complex, located in the mitochondrial inner membrane. It is also known as Complex I and is the largest of the five complexes of the electron transport chain.^[6]

Gene

The NDUFB11 gene is located on the p arm of chromosome X in position 11.23 and is 2,994 base pairs long.^[7][8]

Protein

The NDUFB11 protein weighs 17 kDa and is composed of 153 amino acids.^[7][8] NDUFB11 is a subunit of the enzyme NADH dehydrogenase (ubiquinone), the largest of the respiratory complexes.

Structure

The structure is L-shaped with a long, hydrophobic transmembrane domain and a hydrophilic domain for the peripheral arm that includes all the known redox centers and the NADH binding site.^[6] It has been noted that the N-terminal hydrophobic domain has the potential to be folded into an alpha helix spanning the inner mitochondrial membrane with a C-terminal hydrophilic domain interacting with globular subunits of Complex I. The highly conserved two-domain structure suggests that this feature is critical for the protein function and that the hydrophobic domain acts as an anchor for the NADH dehydrogenase (ubiquinone) complex at the inner mitochondrial membrane.^[5]

Function

The protein encoded by this gene is an accessory subunit of the multisubunit NADH:ubiquinone oxidoreductase (complex I) that is not directly involved in catalysis. Mammalian complex I is composed of 45 different subunits. It locates at the mitochondrial inner membrane. This protein complex has NADH dehydrogenase activity and oxidoreductase activity. It transfers electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. Alternative splicing occurs at this locus and two transcript variants encoding distinct isoforms have been identified.^[5] Initially, NADH binds to Complex I and transfers two electrons to the isoalloxazine ring of the flavin mononucleotide (FMN) prosthetic arm to form FMNH₂. The electrons are transferred through a series of iron-sulfur (Fe-S) clusters in the prosthetic arm and finally to coenzyme Q10 (CoQ), which is reduced to ubiquinol (CoQH₂). The flow of electrons changes the redox state of the protein, resulting in a conformational change and pK shift of the ionizable side chain, which pumps four hydrogen ions out of the mitochondrial matrix.^[6]

References

Further reading