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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
MeSH Review

Protein Deficiency

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Disease relevance of Protein Deficiency


High impact information on Protein Deficiency

  • Yeast knockout models as well as histological and biochemical data from heart biopsies or autopsies of FRDA patients have shown that frataxin defects cause a specific iron-sulfur protein deficiency and intramitochondrial iron accumulation [6].
  • Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia [7].
  • The occurrence of distinct DNA markers in patients from unrelated kinships indicates that, like forms of the condition displaying dysfunctional C1 inhibitor variants, those characterized by protein deficiency are also genetically heterogeneous [8].
  • Here, we have used gene silencing to assess the effect of SMN protein deficiency on U snRNP metabolism in living cells and organisms [9].
  • Genetic cholesteryl ester transfer protein deficiency caused by two prevalent mutations as a major determinant of increased levels of high density lipoprotein cholesterol [10].

Chemical compound and disease context of Protein Deficiency


Biological context of Protein Deficiency


Anatomical context of Protein Deficiency


Gene context of Protein Deficiency

  • Accumulation of apolipoprotein E-rich high density lipoproteins in hyperalphalipoproteinemic human subjects with plasma cholesteryl ester transfer protein deficiency [26].
  • In both mutants, reduced amounts of spliced petB RNAs (encoding the cytochrome b(6) subunit) were detected, thus explaining the observed protein deficiencies [27].
  • We have used an in vivo tumor model to evaluate the consequences of p53 tumor suppressor protein deficiency in a tissue-specific context [28].
  • Genomic and mutational analysis of the mitochondrial trifunctional protein beta-subunit (HADHB) gene in patients with trifunctional protein deficiency [29].
  • Moreover, the deletion strains did not exhibit the pleiotropic membrane protein deficiency observed with certain envZ mutants [30].

Analytical, diagnostic and therapeutic context of Protein Deficiency


  1. Effect of stanozolol in patients with pityriasis rubra pilaris and retinol-binding protein deficiency. Bergamaschini, L., Tucci, A., Colombo, A., Agostoni, A., Finzi, A.G., Altomare, G.F., Pigatto, P.D. N. Engl. J. Med. (1982) [Pubmed]
  2. Stable delivery of physiologic levels of recombinant erythropoietin to the systemic circulation by intramuscular injection of replication-defective adenovirus. Tripathy, S.K., Goldwasser, E., Lu, M.M., Barr, E., Leiden, J.M. Proc. Natl. Acad. Sci. U.S.A. (1994) [Pubmed]
  3. Calpain-3 and dysferlin protein screening in patients with limb-girdle dystrophy and myopathy. Fanin, M., Pegoraro, E., Matsuda-Asada, C., Brown, R.H., Angelini, C. Neurology (2001) [Pubmed]
  4. Neonatal lupus erythematosus syndrome: analysis of C4 allotypes and C4 genes in 18 families. Watson, R.M., Scheel, J.N., Petri, M., Lee, L.A., Bias, W.B., McLean, R.H. Medicine (Baltimore) (1992) [Pubmed]
  5. Dietary protein deficiency and Mycobacterium bovis BCG affect interleukin-2 activity in experimental pulmonary tuberculosis. McMurray, D.N., Mintzer, C.L., Bartow, R.A., Parr, R.L. Infect. Immun. (1989) [Pubmed]
  6. Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits. Puccio, H., Simon, D., Cossée, M., Criqui-Filipe, P., Tiziano, F., Melki, J., Hindelang, C., Matyas, R., Rustin, P., Koenig, M. Nat. Genet. (2001) [Pubmed]
  7. Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia. Rötig, A., de Lonlay, P., Chretien, D., Foury, F., Koenig, M., Sidi, D., Munnich, A., Rustin, P. Nat. Genet. (1997) [Pubmed]
  8. Altered C1 inhibitor genes in type I hereditary angioedema. Stoppa-Lyonnet, D., Tosi, M., Laurent, J., Sobel, A., Lagrue, G., Meo, T. N. Engl. J. Med. (1987) [Pubmed]
  9. Reduced U snRNP assembly causes motor axon degeneration in an animal model for spinal muscular atrophy. Winkler, C., Eggert, C., Gradl, D., Meister, G., Giegerich, M., Wedlich, D., Laggerbauer, B., Fischer, U. Genes Dev. (2005) [Pubmed]
  10. Genetic cholesteryl ester transfer protein deficiency caused by two prevalent mutations as a major determinant of increased levels of high density lipoprotein cholesterol. Inazu, A., Jiang, X.C., Haraki, T., Yagi, K., Kamon, N., Koizumi, J., Mabuchi, H., Takeda, R., Takata, K., Moriyama, Y. J. Clin. Invest. (1994) [Pubmed]
  11. Reversible hepatotoxicity associated with hepatic vitamin A accumulation in a protein-deficient patient. Weber, F.L., Mitchell, G.E., Powell, D.E., Reiser, B.J., Banwell, J.G. Gastroenterology (1982) [Pubmed]
  12. Plasma amino acid abnormalities in the alcoholic: respective role of alcohol, nutrition, and liver injury. Shaw, S., Lieber, C.S. Gastroenterology (1978) [Pubmed]
  13. Capturing protein interactions in the secretory pathway of living cells. Nyfeler, B., Michnick, S.W., Hauri, H.P. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
  14. Identification of a heritable deficiency of the folate-dependent enzyme 10-formyltetrahydrofolate dehydrogenase in mice. Champion, K.M., Cook, R.J., Tollaksen, S.L., Giometti, C.S. Proc. Natl. Acad. Sci. U.S.A. (1994) [Pubmed]
  15. The combined effects of protein deficiency and chronic ethanol administration on rat ethanol metabolism. Wilson, J.S., Korsten, M.A., Lieber, C.S. Hepatology (1986) [Pubmed]
  16. Apaf-1 protein deficiency confers resistance to cytochrome c-dependent apoptosis in human leukemic cells. Jia, L., Srinivasula, S.M., Liu, F.T., Newland, A.C., Fernandes-Alnemri, T., Alnemri, E.S., Kelsey, S.M. Blood (2001) [Pubmed]
  17. Characterization of regions in hsMAD1 needed for binding hsMAD2. A polymorphic change in an hsMAD1 leucine zipper affects MAD1-MAD2 interaction and spindle checkpoint function. Iwanaga, Y., Kasai, T., Kibler, K., Jeang, K.T. J. Biol. Chem. (2002) [Pubmed]
  18. Degradation of membrane-bound ganglioside GM1. Stimulation by bis(monoacylglycero)phosphate and the activator proteins SAP-B and GM2-AP. Wilkening, G., Linke, T., Uhlhorn-Dierks, G., Sandhoff, K. J. Biol. Chem. (2000) [Pubmed]
  19. Isoniazid tumorigenicity in mice under different experimental conditions. Bhide, S.V., Maru, G.B., Sawai, M.M., Ranadive, K.J. Int. J. Cancer (1978) [Pubmed]
  20. Maternal dietary protein deficiency decreases nitric oxide synthase and ornithine decarboxylase activities in placenta and endometrium of pigs during early gestation. Wu, G., Pond, W.G., Flynn, S.P., Ott, T.L., Bazer, F.W. J. Nutr. (1998) [Pubmed]
  21. Generalized lysosome-associated membrane protein-2 defect explains multisystem clinical involvement and allows leukocyte diagnostic screening in danon disease. Fanin, M., Nascimbeni, A.C., Fulizio, L., Spinazzi, M., Melacini, P., Angelini, C. Am. J. Pathol. (2006) [Pubmed]
  22. Brain histidine and food intake in rats fed diets deficient in single amino acids. Mercer, L.P., Dodds, S.J., Schweisthal, M.R., Dunn, J.D. J. Nutr. (1989) [Pubmed]
  23. Protein deficiency impairs erythropoiesis in rats by reducing serum erythropoietin concentration and the population size of erythroid precursor cells. Okano, M., Ohnota, H., Sasaki, R. J. Nutr. (1992) [Pubmed]
  24. The effects of protein deficiency and fluoride on bone mineral content of rat tibia. Likimani, S., Whitford, G.M., Kunkel, M.E. Calcif. Tissue Int. (1992) [Pubmed]
  25. The effect of maternal protein deficiency during pregnancy and lactation on glucose tolerance and pancreatic islet function in adult rat offspring. Wilson, M.R., Hughes, S.J. J. Endocrinol. (1997) [Pubmed]
  26. Accumulation of apolipoprotein E-rich high density lipoproteins in hyperalphalipoproteinemic human subjects with plasma cholesteryl ester transfer protein deficiency. Yamashita, S., Sprecher, D.L., Sakai, N., Matsuzawa, Y., Tarui, S., Hui, D.Y. J. Clin. Invest. (1990) [Pubmed]
  27. HCF152, an Arabidopsis RNA binding pentatricopeptide repeat protein involved in the processing of chloroplast psbB-psbT-psbH-petB-petD RNAs. Meierhoff, K., Felder, S., Nakamura, T., Bechtold, N., Schuster, G. Plant Cell (2003) [Pubmed]
  28. Increased tumor proliferation and genomic instability without decreased apoptosis in MMTV-ras mice deficient in p53. Hundley, J.E., Koester, S.K., Troyer, D.A., Hilsenbeck, S.G., Subler, M.A., Windle, J.J. Mol. Cell. Biol. (1997) [Pubmed]
  29. Genomic and mutational analysis of the mitochondrial trifunctional protein beta-subunit (HADHB) gene in patients with trifunctional protein deficiency. Orii, K.E., Aoyama, T., Wakui, K., Fukushima, Y., Miyajima, H., Yamaguchi, S., Orii, T., Kondo, N., Hashimoto, T. Hum. Mol. Genet. (1997) [Pubmed]
  30. Isolation and characterization of delta ompB strains of Escherichia coli by a general method based on gene fusions. Garrett, S., Taylor, R.K., Silhavy, T.J., Berman, M.L. J. Bacteriol. (1985) [Pubmed]
  31. Protein deficiency in premature infants receiving parenteral nutrition. Shulman, R.J., DeStefano-Laine, L., Petitt, R., Rahman, S., Reed, T. Am. J. Clin. Nutr. (1986) [Pubmed]
  32. Production of IL2 and IL3 in syngeneic mixed lymphocyte reactions of BALB/c mice are elevated during a period of moderate dietary protein deficiency. Petro, T.M., Schwartz, K.M., Chen, S.S. Immunol. Invest. (1994) [Pubmed]
  33. Identification of genes enhanced by protein-calorie malnutrition by differential display polymerase chain reaction (expression of fibrinogen B beta chain, B cell translocation gene 1 and thyroid hormone responsive protein genes). Lee, A.K., Kang, K.W., Kim, Y.G., Cho, M.K., Lee, M.G., Shim, C.K., Chung, S.J., Kim, S.G. Mol. Cell. Biochem. (2002) [Pubmed]
  34. Vitamin and trace metal levels in recessive dystrophic epidermolysis bullosa. Ingen-Housz-Oro, S., Blanchet-Bardon, C., Vrillat, M., Dubertret, L. Journal of the European Academy of Dermatology and Venereology : JEADV. (2004) [Pubmed]
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