ID:NDUAD_HUMAN DESCRIPTION: RecName: Full=NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13; AltName: Full=Cell death regulatory protein GRIM-19; AltName: Full=Complex I-B16.6; Short=CI-B16.6; AltName: Full=Gene associated with retinoic and interferon-induced mortality 19 protein; Short=GRIM-19; Short=Gene associated with retinoic and IFN-induced mortality 19 protein; AltName: Full=NADH-ubiquinone oxidoreductase B16.6 subunit; 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. SUBUNIT: Complex I is composed of 45 different subunits. Interacts with CARD15, but not with CARD4. Interacts with STAT3, but not with STAT1, STAT2 and STAT5A. Interacts with HHV-8 IRF1, in the nucleus, with HPV-16 E6 and SV40 LT. Interacts with OLFM4. INTERACTION: O43464:HTRA2; NbExp=6; IntAct=EBI-372742, EBI-517086; SUBCELLULAR LOCATION: Mitochondrion inner membrane; Single-pass membrane protein; Matrix side. Nucleus. Note=May be translocated into the nucleus upon IFN/RA treatment. TISSUE SPECIFICITY: Widely expressed, with highest expression in heart, skeletal muscle, liver, kidney and placenta. In intestinal mucosa, down-regulated in areas involved in Crohn disease and ulcerative colitis. DEVELOPMENTAL STAGE: Expressed in numerous fetal tissues. INDUCTION: By IFNB1/IFN-beta combined with all-trans-retinoic acid (ATRA). DISEASE: Defects in NDUFA13 may be a cause of susceptibility to Hurthle cell thyroid carcinoma (HCTC) [MIM:607464]. Hurthle cell thyroid carcinoma accounts for approximately 3% of all thyroid cancers. Although they are classified as variants of follicular neoplasms, they are more often multifocal and somewhat more aggressive and are less likely to take up iodine than are other follicular neoplasms. SIMILARITY: Belongs to the complex I NDUFA13 subunit family. SEQUENCE CAUTION: Sequence=AAD27748.1; Type=Erroneous initiation; Sequence=AAG44670.1; Type=Erroneous initiation; Sequence=AAH00589.2; Type=Erroneous initiation;
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on Q9P0J0
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Gene Ontology (GO) Annotations with Structured Vocabulary
Molecular Function: GO:0003954 NADH dehydrogenase activity GO:0005515 protein binding GO:0005524 ATP binding GO:0008137 NADH dehydrogenase (ubiquinone) activity
Biological Process: GO:0006915 apoptotic process GO:0010952 positive regulation of peptidase activity GO:0030308 negative regulation of cell growth GO:0032981 mitochondrial respiratory chain complex I assembly GO:0035458 cellular response to interferon-beta GO:0043280 positive regulation of cysteine-type endopeptidase activity involved in apoptotic process GO:0045039 protein import into mitochondrial inner membrane GO:0045732 positive regulation of protein catabolic process GO:0045892 negative regulation of transcription, DNA-templated GO:0055114 oxidation-reduction process GO:0071300 cellular response to retinoic acid GO:0072593 reactive oxygen species metabolic process GO:0097190 apoptotic signaling pathway GO:0097191 extrinsic apoptotic signaling pathway GO:2001243 negative regulation of intrinsic apoptotic signaling pathway
AF261134 - Homo sapiens CDA016 mRNA, complete cds. AB528693 - Synthetic construct DNA, clone: pF1KB6924, Homo sapiens NDUFA13 gene for NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 13, without stop codon, in Flexi system. BC009189 - Homo sapiens NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 13, mRNA (cDNA clone MGC:15163 IMAGE:3957082), complete cds. AK298891 - Homo sapiens cDNA FLJ58045 complete cds, highly similar to NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 13 (EC 1.6.5.3). AK293859 - Homo sapiens cDNA FLJ57958 complete cds, highly similar to NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 13 (EC 1.6.5.3). AK293965 - Homo sapiens cDNA FLJ59191 complete cds, highly similar to NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 13 (EC 1.6.5.3). BC000589 - Homo sapiens NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 13, mRNA (cDNA clone IMAGE:3346444), partial cds. AF286697 - Homo sapiens novel cell death-regulatory protein GRIM19 (GRIM19) mRNA, complete cds. AF155662 - Homo sapiens putative 16.7 kDa protein mRNA, complete cds. KJ893803 - Synthetic construct Homo sapiens clone ccsbBroadEn_03197 NDUFA13 gene, encodes complete protein. KR710367 - Synthetic construct Homo sapiens clone CCSBHm_00011956 NDUFA13 (NDUFA13) mRNA, encodes complete protein. AF132973 - Homo sapiens CGI-39 protein mRNA, complete cds. CQ903807 - Sequence 9 from Patent WO2004094661.
Biochemical and Signaling Pathways
BioCyc Knowledge Library PWY-3781 - aerobic respiration I (cytochrome c)
Reactome (by CSHL, EBI, and GO)
Protein Q9P0J0 (Reactome details) participates in the following event(s):
R-HSA-6799178 Intermediate 1 binds HP subcomplex to form Intermediate 2 R-HSA-6799191 Intermediate 2 binds MT-ND1:NDUFAF5:NDUFAF6 to form a 315kDa subcomplex R-HSA-6799202 The 315kDa subcomplex binds the 370kDa subcomplex to form the 550kDa complex R-HSA-6799197 ND4, ND5 bind the 550kDa complex to form the 815kDa complex R-HSA-6799179 Peripheral arm subunits bind the 815kDa complex to form a 980kDa complex R-HSA-6799196 The MCIA complex, NDUFAF2-7 all dissociate from the 980kDa complex, resulting in Complex I R-HSA-163217 Complex I oxidises NADH to NAD+, reduces CoQ to QH2 R-HSA-6799198 Complex I biogenesis R-HSA-611105 Respiratory electron transport R-HSA-163200 Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. R-HSA-1428517 The citric acid (TCA) cycle and respiratory electron transport R-HSA-1430728 Metabolism
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
