NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial
Also known as: NDUFB11
Function
Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
Classification
- Family (Pfam)
- PF10183 ESSS
- InterPro
- NADH_UbQ_OxRdtase_ESSS_su
- Functional cluster
- Membrane Channels & Lipid-Anchored Proteins
Experimental structures · PDB · 7
A predicted model is available from AlphaFold.
Gene Ontology · 6
Disease associations
- linear skin defects with multiple congenital anomalies 3 MONDO:0010494
- mitochondrial complex I deficiency, nuclear type 30 MONDO:0026721
Drugs targeting this protein · 2
- METFORMIN HYDROCHLORIDE inhibitor
- ME-344 inhibitor
Related proteins · sequence + function similarity
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial 1.00
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial 0.99
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial 0.98
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial 0.97
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial 0.94
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial 0.94
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial 0.79
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial 0.79
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial 0.78
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial 0.77
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial 0.77
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial 0.76
Co-cited proteins · studied together in the literature
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial 2 shared papers
- Mitochondrial import receptor subunit TOM40 homolog 1 shared papers
- B-cell receptor-associated protein 31 1 shared papers
- Protoheme IX farnesyltransferase, mitochondrial 1 shared papers
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial 2 shared papers
- Non-selective voltage-gated ion channel VDAC1 1 shared papers
- NADH dehydrogenase (ubiquinone) complex I, assembly factor 6 1 shared papers
- Apoptosis regulator Bcl-2 1 shared papers
- Nondiscriminating glutamyl-tRNA synthetase EARS2, mitochondrial 1 shared papers
- Enoyl-CoA hydratase, mitochondrial 1 shared papers
- 5-taurinomethyluridine-[tRNA] synthase subunit GTPB3, mitochondrial 1 shared papers
- Small ribosomal subunit protein mS23 1 shared papers
Literature · 14 cited papers
- BAP31 regulates mitochondrial function via interaction with Tom40 within ER-mitochondria contact sites. Sci. Adv. · 2019
- Accessory subunits are integral for assembly and function of human mitochondrial complex I. Nature · 2016
- A comprehensive genomic analysis reveals the genetic landscape of mitochondrial respiratory chain complex deficiencies. PLoS Genet. · 2016
- N-terminome analysis of the human mitochondrial proteome. Proteomics · 2015
- Exome sequencing of patients with histiocytoid cardiomyopathy reveals a de novo NDUFB11 mutation that plays a role in the pathogenesis of histiocytoid cardiomyopathy. Am. J. Med. Genet. A · 2015
- Mutations in NDUFB11, encoding a complex I component of the mitochondrial respiratory chain, cause microphthalmia with linear skin defects syndrome. Am. J. Hum. Genet. · 2015
- An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J. Proteomics · 2014
- Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
- The DNA sequence of the human X chromosome. Nature · 2005
- The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
- Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat. Genet. · 2004
- The secreted protein discovery initiative (SPDI), a large-scale effort to identify novel human secreted and transmembrane proteins: a bioinformatics assessment. Genome Res. · 2003
- … and 2 more in the literature graph
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