NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9

UniProt Q9Y6M9 Organism Homo sapiens Gene LYRM3, NDUFB9, UQOR22

Also known as: LYRM3, NDUFB9, UQOR22

Function

Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed to be not 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): PF05347 Complex1_LYR
InterPro: Complex1_LYR_dom, Complex1_LYR_NDUFB9_LYRM3, NDUFB9
Functional cluster: RuBisCO & Carbon-Fixation Enzymes

Experimental structures · PDB · 7

5XTC EM 3.70A
5XTD EM 3.70A
5XTH EM 3.90A
5XTI EM 17.40A
9CWT EM 3.44A
9I4I EM 2.63A
9TI4 EM 2.66A

A predicted model is available from AlphaFold.

Gene Ontology · 8

Disease associations

mitochondrial complex I deficiency, nuclear type 24 MONDO:0032628

Drugs targeting this protein · 2

METFORMIN HYDROCHLORIDE inhibitor
ME-344 inhibitor

Related proteins · sequence + function similarity

Co-cited proteins · studied together in the literature

Thy-1 membrane glycoprotein 1 shared papers
ATP-binding cassette sub-family F member 2 1 shared papers
Calpain-2 catalytic subunit 1 shared papers
Glyceraldehyde-3-phosphate dehydrogenase 1 shared papers
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 1 shared papers
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 1 shared papers
Distal membrane-arm assembly complex protein 1 1 shared papers
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 1 shared papers
NF-kappa-B essential modulator 1 shared papers
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 1 shared papers
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial 1 shared papers
NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial 1 shared papers

Literature · 12 cited papers

Accessory subunits are integral for assembly and function of human mitochondrial complex I. Nature · 2016
N-terminome analysis of the human mitochondrial proteome. Proteomics · 2015
An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J. Proteomics · 2014
Mutation screening of 75 candidate genes in 152 complex I deficiency cases identifies pathogenic variants in 16 genes including NDUFB9. J. Med. Genet. · 2012
Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
Kinase-selective enrichment enables quantitative phosphoproteomics of the kinome across the cell cycle. Mol. Cell · 2008
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 subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification. J. Biol. Chem. · 2003
Cloning and functional analysis of cDNAs with open reading frames for 300 previously undefined genes expressed in CD34+ hematopoietic stem/progenitor cells. Genome Res. · 2000
cDNA cloning by amplification of circularized first strand cDNAs reveals non-IRE-regulated iron-responsive mRNAs. Biochem. Biophys. Res. Commun. · 2000
Human NDUFB9 gene: genomic organization and a possible candidate gene associated with deafness disorder mapped to chromosome 8q13. Hum. Hered. · 1999

A document in the lmmol reference corpus — open public data (UniProt, GO, PDB, the literature graph) rendered as a single cross-linked page. Hover any link to preview its target.