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Gene Review

NDUFA5  -  NADH dehydrogenase (ubiquinone) 1 alpha...

Homo sapiens

Synonyms: B13, CI-13KD-B, CI-13kB, CI-13kD-B, Complex I subunit B13, ...
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Disease relevance of NDUFA5

  • Autoimmunity in Chagas' disease. Identification of cardiac myosin-B13 Trypanosoma cruzi protein crossreactive T cell clones in heart lesions of a chronic Chagas' cardiomyopathy patient [1].
  • Analysis of leukemic cells from four other patients with breakpoint cluster region-unrearranged Philadelphia chromosome-positive acute lymphoblastic leukemia revealed a rearrangement on chromosome 22 close to the breakpoint in SUP-B13 in only one patient [2].
  • Therefore, B13 and related analogues of ceramide and inhibitors of ceramidases offer a promising therapeutic strategy with selective toxicity toward malignant but not normal cells [3].
  • Rocket and two-dimensional immunoelectrophoreses were used to demonstrate that antisera from rabbits immunized with Streptococcus mutans strain B13 cross-reacted with human heart tissue [4].
  • Substituted muconic acids were prepared from the corresponding catechols by pyrocatechase II from Pseudomonas sp. B13 [5].

High impact information on NDUFA5

  • We recently identified a heart-specific epitope of cardiac myosin heavy chain (residues 1442-1447, AAALDK) that is crossreactive with a homologous sequence (AAAGDK) of the immunodominant T. cruzi antigen B13 [1].
  • In this paper, we obtained heart-infiltrating T cell clones from CCC patients to assess whether molecular mimicry between cardiac myosin and B13 is directly involved in the genesis of heart lesions [1].
  • In the mouse cell assays [using bone marrow or the pro-B cell line (B13)] the hybrids clearly identified the importance of residues in the C terminus for biological activity [6].
  • Lowering the pH to 5.7 in 1 M Na2SO4 without dihaloalkanes induces a cooperative structural transition in which the dyad cavities between B13 glutamate pairs are constricted, and SO4(2-) ions are bound by rearranged triads of B1 NH+3 groups [7].
  • To characterize the abnormality in the breakpoint cluster region-unrearranged form, we have mapped a 9;22 translocation from the Philadelphia chromosome-positive acute lymphoblastic leukemia cell line SUP-B13 by using pulsed-field gel electrophoresis and have cloned the DNA at the translocation junctions [2].

Chemical compound and disease context of NDUFA5

  • Resting cells of most S. sanguis strains bound extracellular GTase from Streptococcus mutans strain B13 (serotype d), resulting in the strong adherence of the S. sanguis cells to smooth glass surfaces in the presence of sucrose [8].
  • Although the cis,cis-configuration of 2-fluoromuconic acid was confirmed by corresponding spectroscopic data, it was not cycloisomerized by crude extracts or cycloisomerase II preparations from Pseudomonas sp. B13 [5].
  • It was most notable that the expression levels of HOXA1, A2, C4 and B13 in melanoma with distant metastasis were higher than those in melanoma without it [9].
  • In in vivo studies using a model of xenografted androgen-insensitive prostate cancer, B13 sensitized the tumors to the effects of radiation, resulting in a significant reduction in tumor volume and weight after treatment with the combination of B13 and radiation [10].
  • The aim of our study was to investigate the effect of resin composite components on glucan synthesis by glucosyltransferase (GTase) derived from a cariogenic bacterium, Streptococcus sobrinus B13 [11].

Biological context of NDUFA5


Anatomical context of NDUFA5

  • Human B13 mRNA expression was observed in all tissues examined with highest levels in heart, skeletal muscle and brain [14].
  • The real-time RT-PCR analysis revealed that the expressions of the NDUFA5 mRNA, but not the ZNF9 mRNA level, were significantly up-regulated in all the tested cell lines derived from HPV-positive cervical cancer, HeLa, SW576, and CaSKi [17].
  • The highly efficient retroviral cDNA expression system derived from HeLa cells identified two cDNA species coding NADH dehydrogenase 1 alpha subcomplex 5 (NDUFA5) and zinc finger protein 9 (ZNF9), exhibiting the potential to transform murine fibroblast cell line, NIH3T3 [17].
  • These results indicate that the B13 progenitor cell model is suitable for studying liver function and for understanding the molecular and cellular events that occur during transdifferentiation [18].
  • The B13 cell markers amylase, synaptophysin, and neurofilament were lost in transdifferentiated hepatocytes compared to control cells and the liver enriched transcription factors C/EBPbeta and C/EBPalpha were induced first, followed by HNF4alpha and then RXRalpha [18].

Associations of NDUFA5 with chemical compounds

  • The derivative in which the two arginines in positions B13 and B17 had been replaced by the uncharged isosteric amino acid citrulline were biologically inactive [19].
  • Two arginine residues in positions B13 and B17 that project like forefinger and middle finger from the helix provide the electrostatic element opposed by the hydrophobic (thumb) element isoleucine (B20), offset from the arginines by about 40 degrees [20].
  • Arginines B13 and B17 are each chelated by an aspartic acid/glutamic acid pair and by isoleucine B20, which, offset by a one-quarter helix turn from a straight line connecting the arginines, interacts with a cluster of hydrophobic amino acids [21].
  • The assembly of the insulin hexamer brings the six B13 glutamate side-chains at the centre into close proximity [22].
  • Hepatic transdifferentiation was induced in 11.5-day mouse embryonic pancreas or the pancreatic cell line AR42J-B13 (B13) by culture with dexamethasone [23].

Analytical, diagnostic and therapeutic context of NDUFA5

  • The ceramide analog, B13, induces apoptosis in prostate cancer cell lines and inhibits tumor growth in prostate cancer xenografts [10].
  • The median percentage inhibition of B13 ELISA for peptide S12 was 94%, while those of the RI analogue or the other topological analogues were below 12% [24].
  • We have applied a double determinant immunoassay (DDIA) to HLA-A2,A28, and B13 typing, using serum as an antigen source [25].
  • The results obtained show a correlation of 96% (B13) and 89.1% (A2,A28) with the results obtained by conventional HLA typing [25].
  • Ethnic differences in B13 molecules were identified by one-dimensional isoelectric focusing in which the pI of desialated Oriental B13 molecules was found to be higher than that of Caucasians [26].


  1. Autoimmunity in Chagas' disease. Identification of cardiac myosin-B13 Trypanosoma cruzi protein crossreactive T cell clones in heart lesions of a chronic Chagas' cardiomyopathy patient. Cunha-Neto, E., Coelho, V., Guilherme, L., Fiorelli, A., Stolf, N., Kalil, J. J. Clin. Invest. (1996) [Pubmed]
  2. Heterogeneity of genomic fusion of BCR and ABL in Philadelphia chromosome-positive acute lymphoblastic leukemia. Rubin, C.M., Carrino, J.J., Dickler, M.N., Leibowitz, D., Smith, S.D., Westbrook, C.A. Proc. Natl. Acad. Sci. U.S.A. (1988) [Pubmed]
  3. Induction of apoptotic cell death and prevention of tumor growth by ceramide analogues in metastatic human colon cancer. Selzner, M., Bielawska, A., Morse, M.A., Rüdiger, H.A., Sindram, D., Hannun, Y.A., Clavien, P.A. Cancer Res. (2001) [Pubmed]
  4. Cross-reactivity of Streptococcus mutans antigens and human heart tissue. Ferretti, J.J., Shea, C., Humphrey, M.W. Infect. Immun. (1980) [Pubmed]
  5. Chemical structure and biodegradability of halogenated aromatic compounds. Halogenated muconic acids as intermediates. Schmidt, E., Remberg, G., Knackmuss, H.J. Biochem. J. (1980) [Pubmed]
  6. Structure-function analysis of interleukin-5 utilizing mouse/human chimeric molecules. McKenzie, A.N., Barry, S.C., Strath, M., Sanderson, C.J. EMBO J. (1991) [Pubmed]
  7. Stereospecific dihaloalkane binding in a pH-sensitive cavity in cubic insulin crystals. Gursky, O., Fontano, E., Bhyravbhatla, B., Caspar, D.L. Proc. Natl. Acad. Sci. U.S.A. (1994) [Pubmed]
  8. Adherence of Streptococcus sanguis clinical isolates to smooth surfaces and interactions of the isolates with Streptococcus mutans glucosyltransferase. Hamada, S., Torii, M., Kotani, S., Tsuchitani, Y. Infect. Immun. (1981) [Pubmed]
  9. Altered expressions of HOX genes in human cutaneous malignant melanoma. Maeda, K., Hamada, J., Takahashi, Y., Tada, M., Yamamoto, Y., Sugihara, T., Moriuchi, T. Int. J. Cancer (2005) [Pubmed]
  10. The ceramide analog, B13, induces apoptosis in prostate cancer cell lines and inhibits tumor growth in prostate cancer xenografts. Samsel, L., Zaidel, G., Drumgoole, H.M., Jelovac, D., Drachenberg, C., Rhee, J.G., Brodie, A.M., Bielawska, A., Smyth, M.J. Prostate (2004) [Pubmed]
  11. Effects of resin composite components on glucosyltransferase of cariogenic bacterium. Kawai, K., Tsuchitani, Y. J. Biomed. Mater. Res. (2000) [Pubmed]
  12. Genomic organization of the human complex I 13-kDa subunit gene NDUFA5. Tensing, K., Pata, I., Wittig, I., Wehnert, M., Metspalu, A. Cytogenet. Cell Genet. (1999) [Pubmed]
  13. Cloning of the human NADH: ubiquinone oxidoreductase subunit B13: localization to chromosome 7q32 and identification of a pseudogene on 11p15. Russell, M.W., du Manoir, S., Collins, F.S., Brody, L.C. Mamm. Genome (1997) [Pubmed]
  14. A human cDNA encoding the homologue of NADH: ubiquinone oxidoreductase subunit B13. Pata, I., Tensing, K., Metspalu, A. Biochim. Biophys. Acta (1997) [Pubmed]
  15. Ceramide-dependent regulation of human epidermal keratinocyte CD1d expression during terminal differentiation. Fishelevich, R., Malanina, A., Luzina, I., Atamas, S., Smyth, M.J., Porcelli, S.A., Gaspari, A.A. J. Immunol. (2006) [Pubmed]
  16. Epigenetic inactivation of the candidate tumor suppressor gene HOXB13 in human renal cell carcinoma. Okuda, H., Toyota, M., Ishida, W., Furihata, M., Tsuchiya, M., Kamada, M., Tokino, T., Shuin, T. Oncogene (2006) [Pubmed]
  17. Identification of NADH dehydrogenase 1 alpha subcomplex 5 capable to transform murine fibroblasts and overexpressed in human cervical carcinoma cell lines. Shimada, T., Moriuchi, R., Mori, T., Yamada, K., Ishimaru, T., Katamine, S. Biochem. Biophys. Res. Commun. (2006) [Pubmed]
  18. Characterization of liver function in transdifferentiated hepatocytes. Burke, Z.D., Shen, C.N., Ralphs, K.L., Tosh, D. J. Cell. Physiol. (2006) [Pubmed]
  19. Total synthesis of human relaxin and human relaxin derivatives by solid-phase peptide synthesis and site-directed chain combination. Büllesbach, E.E., Schwabe, C. J. Biol. Chem. (1991) [Pubmed]
  20. The relaxin receptor-binding site geometry suggests a novel gripping mode of interaction. Büllesbach, E.E., Schwabe, C. J. Biol. Chem. (2000) [Pubmed]
  21. The trap-like relaxin-binding site of the leucine-rich G-protein-coupled receptor 7. Büllesbach, E.E., Schwabe, C. J. Biol. Chem. (2005) [Pubmed]
  22. Role of B13 Glu in insulin assembly. The hexamer structure of recombinant mutant (B13 Glu-->Gln) insulin. Bentley, G.A., Brange, J., Derewenda, Z., Dodson, E.J., Dodson, G.G., Markussen, J., Wilkinson, A.J., Wollmer, A., Xiao, B. J. Mol. Biol. (1992) [Pubmed]
  23. Induction and regulation of acute phase proteins in transdifferentiated hepatocytes. Kurash, J.K., Shen, C.N., Tosh, D. Exp. Cell Res. (2004) [Pubmed]
  24. Retro-inverso peptide analogues of Trypanosoma cruzi B13 protein epitopes fail to be recognized by human sera and peripheral blood mononuclear cells. Iwai, L.K., Duranti, M.A., Abel, L.C., Juliano, M.A., Kalil, J., Juliano, L., Cunha-Neto, E. Peptides (2001) [Pubmed]
  25. A double determinant immunoassay for HLA class I typing using serum as an antigen source. Russo, C., Fotino, M., Carbonara, A., Ferrone, S. Hum. Immunol. (1987) [Pubmed]
  26. IEF patterns of HLA-B13 antigens from Orientals and Caucasians. Yang, S.Y., Chang, A., Olivero, R., Relias, V., Yunis, E.J. Immunogenetics (1985) [Pubmed]
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