NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13

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. Involved in the interferon/all-trans-retinoic acid (IFN/RA) induced cell death. This apoptotic activity is inhibited by interaction with viral IRF1. Prevents the transactivation of STAT3 target genes. May play a role in CARD15-mediated innate mucosal responses and serve to regulate intestinal epithelial cell responses to microbes.

Classification

Family (Pfam)

PF06212 GRIM-19

InterPro

GRIM-19

Functional cluster

Central Metabolic Enzymes (KARI/Glycolysis)

Experimental structures · PDB · 8

• 5XTB EM 3.40A
• 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 · 20

• GO:0005737 cytoplasm
• GO:0005743 mitochondrial inner membrane
• GO:0031966 mitochondrial membrane
• GO:0005739 mitochondrion
• GO:0005654 nucleoplasm
• GO:0098803 respiratory chain complex
• GO:0045271 respiratory chain complex I
• GO:0005524 ATP binding
• GO:0061133 endopeptidase activator activity
• GO:0009060 aerobic respiration
• GO:0006915 apoptotic process
• GO:0035458 cellular response to interferon-beta
• GO:0071300 cellular response to retinoic acid
• GO:0032981 mitochondrial respiratory chain complex I assembly
• GO:0045892 negative regulation of DNA-templated transcription
• GO:1900119 positive regulation of execution phase of apoptosis
• GO:0045732 positive regulation of protein catabolic process
• GO:0045039 protein insertion into mitochondrial inner membrane
• GO:0042776 proton motive force-driven mitochondrial ATP synthesis
• GO:0022904 respiratory electron transport chain

Disease associations

• thyroid Hurthle cell carcinoma MONDO:0011836
• mitochondrial complex I deficiency, nuclear type 28 MONDO:0032632

Drugs targeting this protein · 2

• METFORMIN HYDROCHLORIDE inhibitor
• ME-344 inhibitor

Related proteins · sequence + function similarity

• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 1.00
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 1.00
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 1.00
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 0.97
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 0.96
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 0.94
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13-A 0.90
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13-B 0.89
• Cytochrome c oxidase assembly protein COX16, mitochondrial 0.71
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 0.71
• Cytochrome c oxidase assembly protein COX16, mitochondrial 0.69
• Cytochrome c oxidase subunit 6C 0.69

Co-cited proteins · studied together in the literature

• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 3 shared papers
• Olfactomedin-4 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
• 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
• NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial 1 shared papers
• NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial 1 shared papers

Literature · 18 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
• Mutation in NDUFA13/GRIM19 leads to early onset hypotonia, dyskinesia and sensorial deficiencies, and mitochondrial complex I instability. Hum. Mol. Genet. · 2015
• Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
• Somatic and germline mutation in GRIM-19, a dual function gene involved in mitochondrial metabolism and cell death, is linked to mitochondrion-rich (Hurthle cell) tumours of the thyroid. Br. J. Cancer · 2005
• GRIM-19 interacts with nucleotide oligomerization domain 2 and serves as downstream effector of anti-bacterial function in intestinal epithelial cells. J. Biol. Chem. · 2005
• The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
• GRIM-19, a cell death regulatory protein, is essential for assembly and function of mitochondrial complex I. Mol. Cell. Biol. · 2004
• GW112, a novel antiapoptotic protein that promotes tumor growth. Cancer Res. · 2004
• The DNA sequence and biology of human chromosome 19. Nature · 2004
• Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat. Genet. · 2004
• The cell death regulator GRIM-19 is an inhibitor of signal transducer and activator of transcription 3. Proc. Natl. Acad. Sci. U.S.A. · 2003
• … and 6 more in the literature graph