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Gene Review

NDUFA13  -  NADH dehydrogenase (ubiquinone) 1 alpha...

Homo sapiens

Synonyms: B16.6, CDA016, CGI-39, CI-B16.6, Cell death regulatory protein GRIM-19, ...
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Disease relevance of NDUFA13


High impact information on NDUFA13

  • The cell death regulator GRIM-19 is an inhibitor of signal transducer and activator of transcription 3 [5].
  • Mutational analysis indicates that the transactivation domain of STAT3, especially residue S727, is required for GRIM-19 binding [5].
  • We reexamined the cellular localization of GRIM-19 in various cell types and found its primary localization in the mitochondria [6].
  • GRIM-19 was originally identified as a nuclear protein with apoptotic nature in interferon (IFN)- and all-trans-retinoic acid (RA)-induced tumor cells [6].
  • Our result demonstrates that GRIM-19, a gene product with a specific role in IFN-RA-induced cell death, is a functional component of mitochondrial complex I and is essential for early embryonic development [6].

Biological context of NDUFA13


Anatomical context of NDUFA13


Associations of NDUFA13 with chemical compounds


Physical interactions of NDUFA13

  • Induction of olfactomedin 4 (OLFM4(GW112/hGC-1)) in cancer cells was recently reported to have a novel antiapoptotic action via binding to the potent apoptosis inducer GRIM-19 [10].

Regulatory relationships of NDUFA13

  • We also show that GRIM-19 inhibits the cell-transforming property of viral oncogenic protein viral IFN regulatory factor-1 (vIRF-1) via a physical interaction. mAbs developed in this study should be useful for studying the other physiologic roles of GRIM-19 and serve as a potent tool for studying tumor responses to IFN/RA therapy [11].

Other interactions of NDUFA13


Analytical, diagnostic and therapeutic context of NDUFA13

  • Using the yeast two-hybrid assay, we identified genes associated with retinoid-IFN-induced mortality-19 (GRIM19), which interacts directly with vIRF1, both in vivo and in vitro [3].
  • To reveal its biological role, we generated mice deficient in GRIM-19 by gene targeting [6].


  1. A proteomic analysis reveals the loss of expression of the cell death regulatory gene GRIM-19 in human renal cell carcinomas. Alchanati, I., Nallar, S.C., Sun, P., Gao, L., Hu, J., Stein, A., Yakirevich, E., Konforty, D., Alroy, I., Zhao, X., Reddy, S.P., Resnick, M.B., Kalvakolanu, D.V. Oncogene (2006) [Pubmed]
  2. GRIM-19 interacts with nucleotide oligomerization domain 2 and serves as downstream effector of anti-bacterial function in intestinal epithelial cells. Barnich, N., Hisamatsu, T., Aguirre, J.E., Xavier, R., Reinecker, H.C., Podolsky, D.K. J. Biol. Chem. (2005) [Pubmed]
  3. Viral interferon regulatory factor 1 of Kaposi's sarcoma-associated herpesvirus interacts with a cell death regulator, GRIM19, and inhibits interferon/retinoic acid-induced cell death. Seo, T., Lee, D., Shim, Y.S., Angell, J.E., Chidambaram, N.V., Kalvakolanu, D.V., Choe, J. J. Virol. (2002) [Pubmed]
  4. 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. Máximo, V., Botelho, T., Capela, J., Soares, P., Lima, J., Taveira, A., Amaro, T., Barbosa, A.P., Preto, A., Harach, H.R., Williams, D., Sobrinho-Simões, M. Br. J. Cancer (2005) [Pubmed]
  5. The cell death regulator GRIM-19 is an inhibitor of signal transducer and activator of transcription 3. Zhang, J., Yang, J., Roy, S.K., Tininini, S., Hu, J., Bromberg, J.F., Poli, V., Stark, G.R., Kalvakolanu, D.V. Proc. Natl. Acad. Sci. U.S.A. (2003) [Pubmed]
  6. GRIM-19, a cell death regulatory protein, is essential for assembly and function of mitochondrial complex I. Huang, G., Lu, H., Hao, A., Ng, D.C., Ponniah, S., Guo, K., Lufei, C., Zeng, Q., Cao, X. Mol. Cell. Biol. (2004) [Pubmed]
  7. Chromosomal localization of human GRIM-19, a novel IFN-beta and retinoic acid-activated regulator of cell death. Chidambaram, N.V., Angell, J.E., Ling, W., Hofmann, E.R., Kalvakolanu, D.V. J. Interferon Cytokine Res. (2000) [Pubmed]
  8. Point mutation in GRIM-19: a new genetic lesion in Hurthle cell thyroid carcinomas. Fusco, A., Viglietto, G., Santoro, M. Br. J. Cancer (2005) [Pubmed]
  9. Coupling mitochondrial respiratory chain to cell death: an essential role of mitochondrial complex I in the interferon-beta and retinoic acid-induced cancer cell death. Huang, G., Chen, Y., Lu, H., Cao, X. Cell Death Differ. (2007) [Pubmed]
  10. Olfactomedin 4 promotes S-phase transition in proliferation of pancreatic cancer cells. Kobayashi, D., Koshida, S., Moriai, R., Tsuji, N., Watanabe, N. Cancer Sci. (2007) [Pubmed]
  11. Characterization of monoclonal antibodies against GRIM-19, a novel IFN-beta and retinoic acid-activated regulator of cell death. Hu, J., Angell, J.E., Zhang, J., Ma, X., Seo, T., Raha, A., Hayashi, J., Choe, J., Kalvakolanu, D.V. J. Interferon Cytokine Res. (2002) [Pubmed]
  12. GW112, a novel antiapoptotic protein that promotes tumor growth. Zhang, X., Huang, Q., Yang, Z., Li, Y., Li, C.Y. Cancer Res. (2004) [Pubmed]
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