NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2

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)

PF05047 L51_S25_CI-B8

InterPro

NADH_Ub_cplx-1_asu_su-2, Ribosomal_mL43/mS25/NADH_DH, Thioredoxin-like_sf

Functional cluster

Chloroplast & RuBisCO Photosynthetic Proteins

Experimental structures · PDB · 8

• 1S3A NMR
• 5XTB EM 3.40A
• 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

• GO:0005743 mitochondrial inner membrane
• GO:0031966 mitochondrial membrane
• GO:0005739 mitochondrion
• GO:0045271 respiratory chain complex I
• GO:0008137 NADH dehydrogenase (ubiquinone) activity
• GO:0009060 aerobic respiration
• GO:0006120 mitochondrial electron transport, NADH to ubiquinone
• GO:0042776 proton motive force-driven mitochondrial ATP synthesis

Disease associations

• mitochondrial complex I deficiency, nuclear type 13 MONDO:0032618

Drugs targeting this protein · 2

• METFORMIN HYDROCHLORIDE inhibitor
• ME-344 inhibitor

Related proteins · sequence + function similarity

• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 0.99
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 0.99
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 0.98
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 0.98
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 0.98
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 0.97
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 0.89
• NADH-ubiquinone oxidoreductase 10.5 kDa subunit 0.81
• Large ribosomal subunit protein mL43 0.64
• Large ribosomal subunit protein mL43 0.62
• Large ribosomal subunit protein mL53 0.60
• Large ribosomal subunit protein mL43 0.60

Co-cited proteins · studied together in the literature

• NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial 2 shared papers
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 2 shared papers
• NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial 2 shared papers
• NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 2 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
• 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] 1 beta subcomplex subunit 9 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
• Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
• Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach. Anal. Chem. · 2009
• NDUFA2 complex I mutation leads to Leigh disease. Am. J. Hum. Genet. · 2008
• The consensus coding sequences of human breast and colorectal cancers. Science · 2006
• The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
• The DNA sequence and comparative analysis of human chromosome 5. Nature · 2004
• The oxidized subunit B8 from human complex I adopts a thioredoxin fold. Structure · 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
• Identification and primary structure of five human NADH-ubiquinone oxidoreductase subunits. Biochem. Biophys. Res. Commun. · 1997