NDUFB11

NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 11, 17.3kDa
Identifiers
Aliases NDUFB11, CI-ESSS, ESSS, NP17.3, Np15, P17.3, LSDMCA3
External IDs MGI: 1349919 HomoloGene: 10398 GeneCards: 54539
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
XM_006527554

RefSeq (protein)

NP_001129470.1
NP_061929.2

NP_062308.2
XP_006527617.1

Location (UCSC) Chr X: 47.14 – 47.15 Mb Chr X: 20.62 – 20.62 Mb
PubMed search
Wikidata
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ESSS subunit of NADH:ubiquinone oxidoreductase (complex I)
Identifiers
Symbol ESSS
Pfam PF10183
InterPro IPR019329

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.[1][2][3] 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.[4]

Gene

The NDUFB11 gene is located on the p arm of chromosome X in position 11.23 and is 2,994 base pairs long.[5][6]

Protein

The NDUFB11 protein weighs 17 kDa and is composed of 153 amino acids.[5][6] 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.[4] 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.[3]

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.[3] 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.[4]

References

  1. ↑ Cui Y, Yu L, Gong R, Zhang M, Fan Y, Yue P, Zhao S (Jun 1999). "Cloning and tissue expressional characterization of a full-length cDNA encoding human neuronal protein P17.3". Biochemical Genetics 37 (5-6): 175–85. doi:10.1023/A:1018734605214. PMID 10544803.
  2. ↑ Carroll J, Shannon RJ, Fearnley IM, Walker JE, Hirst J (Dec 2002). "Definition of the nuclear encoded protein composition of bovine heart mitochondrial complex I. Identification of two new subunits". The Journal of Biological Chemistry 277 (52): 50311–7. doi:10.1074/jbc.M209166200. PMID 12381726.
  3. 1 2 3 "Entrez Gene: NDUFB11 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 11, 17.3kDa".
  4. 1 2 3 Voet D, Voet JG, Pratt CW (2013). "Chapter 18". Fundamentals of biochemistry: life at the molecular level (4th ed.). Hoboken, NJ: Wiley. pp. 581–620. ISBN 978-0-470-54784-7.
  5. 1 2 Zong NC, Li H, Li H, Lam MP, Jimenez RC, Kim CS, Deng N, Kim AK, Choi JH, Zelaya I, Liem D, Meyer D, Odeberg J, Fang C, Lu HJ, Xu T, Weiss J, Duan H, Uhlen M, Yates JR, Apweiler R, Ge J, Hermjakob H, Ping P (Oct 2013). "Integration of cardiac proteome biology and medicine by a specialized knowledgebase". Circulation Research 113 (9): 1043–53. doi:10.1161/CIRCRESAHA.113.301151. PMC 4076475. PMID 23965338.
  6. 1 2 "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11". Cardiac Organellar Protein Atlas Knowledgebase (COPaKB).

Further reading


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