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Gene Review

PGAM1  -  phosphoglycerate mutase 1 (brain)

Homo sapiens

Synonyms: BPG-dependent PGAM 1, CDABP0006, HEL-S-35, PGAM-B, PGAMA, ...
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Disease relevance of PGAM1

  • Overlaps of the tryptic peptides were obtained by studying the V8 Staphylococcus aureus protease peptides of the aminoethylated phosphoglycerate mutase isozyme B either by microsequencing or by mass spectrometry [1].
  • Human muscle phosphoglycerate mutase (PGAM-M) deficiency is associated with exercise intolerance, muscle cramps, chronic serum CK elevation, and recurrent episodes of myoglobinuria [2].
  • Hereditary muscle PGAM deficiency has been identified in 9 patients with myopathy [3].
  • Nine patients with McArdle disease, one with the partial glycolytic defect phosphoglycerate mutase deficiency, and nine matched, healthy subjects performed the classic ischemic forearm protocol and an identical protocol without ischemia [4].
  • The biochemical properties and mechanism of catalysis of the novel spore germination protease that degrades small, acid-soluble proteins protecting DNA against damage, a cofactor independent phosphoglycerate mutase, NAD+ synthetase, and the three know B. anthracis toxins, protective antigen, lethal factor, and edema factor are described [5].

High impact information on PGAM1


Chemical compound and disease context of PGAM1


Biological context of PGAM1


Anatomical context of PGAM1

  • No PGAM-B activity was detected in testes of newborn mice, in which only the PGAM-AA isozyme was observed [16].
  • PGAM-B might thus be useful as a marker for germ cell differentiation, along with other germ cell-specific proteins [16].
  • We have previously reported the isolation in pure form of the human erythrocyte phosphoglycerate mutase isozyme B. We now report the sequence of the whole protein and the identification of its N-terminal blocking group [1].
  • Analysis of metabolic activity of one of the tumor cell lines, MCF-7, indicated that PGMtide inhibited glycolytic flux, consistent with in vivo inhibition of PGM [18].
  • Leukocyte activation through chemoattractant receptors leads to Pak activation and transient inhibition of endogenous PGAM-B activity [19].

Associations of PGAM1 with chemical compounds


Other interactions of PGAM1


Analytical, diagnostic and therapeutic context of PGAM1


  1. Sequence of the human erythrocyte phosphoglycerate mutase by microsequencer and mass spectrometry. Blouquit, Y., Calvin, M.C., Rosa, R., Promé, D., Promé, J.C., Pratbernou, F., Cohen-Solal, M., Rosa, J. J. Biol. Chem. (1988) [Pubmed]
  2. Molecular basis of muscle phosphoglycerate mutase (PGAM-M) deficiency in the Italian kindred. Toscano, A., Tsujino, S., Vita, G., Shanske, S., Messina, C., Dimauro, S. Muscle Nerve (1996) [Pubmed]
  3. Molecular genetic studies in muscle phosphoglycerate mutase (PGAM-M) deficiency. Tsujino, S., Shanske, S., Sakoda, S., Toscano, A., DiMauro, S. Muscle Nerve (1995) [Pubmed]
  4. A nonischemic forearm exercise test for McArdle disease. Kazemi-Esfarjani, P., Skomorowska, E., Jensen, T.D., Haller, R.G., Vissing, J. Ann. Neurol. (2002) [Pubmed]
  5. The structure and function of novel proteins of Bacillus anthracis and other spore-forming bacteria: development of novel prophylactic and therapeutic agents. Jedrzejas, M.J. Crit. Rev. Biochem. Mol. Biol. (2002) [Pubmed]
  6. Human muscle phosphoglycerate mutase deficiency: newly discovered metabolic myopathy. DiMauro, S., Miranda, A.F., Khan, S., Gitlin, K., Friedman, R. Science (1981) [Pubmed]
  7. Target discovery in small-molecule cell-based screens by in situ proteome reactivity profiling. Evans, M.J., Saghatelian, A., Sorensen, E.J., Cravatt, B.F. Nat. Biotechnol. (2005) [Pubmed]
  8. The molecular genetic basis of muscle phosphoglycerate mutase (PGAM) deficiency. Tsujino, S., Shanske, S., Sakoda, S., Fenichel, G., DiMauro, S. Am. J. Hum. Genet. (1993) [Pubmed]
  9. A single MEF-2 site is a major positive regulatory element required for transcription of the muscle-specific subunit of the human phosphoglycerate mutase gene in skeletal and cardiac muscle cells. Nakatsuji, Y., Hidaka, K., Tsujino, S., Yamamoto, Y., Mukai, T., Yanagihara, T., Kishimoto, T., Sakoda, S. Mol. Cell. Biol. (1992) [Pubmed]
  10. Phosphoglycerate kinase deficiency: another cause of recurrent myoglobinuria. DiMauro, S., Dalakas, M., Miranda, A.F. Ann. Neurol. (1983) [Pubmed]
  11. Isozyme patterns and protein profiles in neuromuscular disorders. Edwards, Y.H., Tipler, T.D., Morgan-Hughes, J.A., Neerunjun, J.S., Hopkinson, D.A. J. Med. Genet. (1982) [Pubmed]
  12. Phosphoglycerate mutase, 2,3-bisphosphoglycerate phosphatase and enolase activity and isoenzymes in lung, colon and liver carcinomas. Durany, N., Joseph, J., Campo, E., Molina, R., Carreras, J. Br. J. Cancer (1997) [Pubmed]
  13. Resveratrol-induced cell growth inhibition and apoptosis is associated with modulation of phosphoglycerate mutase B in human prostate cancer cells: two-dimensional sodium dodecyl sulfate-polyacrylamide gel electrophoresis and mass spectrometry evaluation. Narayanan, N.K., Narayanan, B.A., Nixon, D.W. Cancer Detect. Prev. (2004) [Pubmed]
  14. The gene for human muscle-specific phosphoglycerate mutase, PGAM2, mapped to chromosome 7 by polymerase chain reaction. Edwards, Y.H., Sakoda, S., Schon, E., Povey, S. Genomics (1989) [Pubmed]
  15. Isolation of a cDNA encoding the B isozyme of human phosphoglycerate mutase (PGAM) and characterization of the PGAM gene family. Sakoda, S., Shanske, S., DiMauro, S., Schon, E.A. J. Biol. Chem. (1988) [Pubmed]
  16. Developmental activation of phosphoglycerate mutase-2 in the testis of the mouse. Fundele, R., Winking, H., Illmensee, K., Jägerbauer, E.M. Dev. Biol. (1987) [Pubmed]
  17. In situ mapping of the muscle-specific form of phosphoglycerate mutase gene to human chromosome 7p12-7p13. Castella-Escola, J., Mattei, M.G., Ojcius, D.M., Passage, E., Valentin, C., Cohen-Solal, M. Hum. Genet. (1990) [Pubmed]
  18. Phosphoglycerate mutase-derived polypeptide inhibits glycolytic flux and induces cell growth arrest in tumor cell lines. Engel, M., Mazurek, S., Eigenbrodt, E., Welter, C. J. Biol. Chem. (2004) [Pubmed]
  19. A p21-activated kinase-controlled metabolic switch up-regulates phagocyte NADPH oxidase. Shalom-Barak, T., Knaus, U.G. J. Biol. Chem. (2002) [Pubmed]
  20. Cloning, purification, crystallization and preliminary crystallographic analysis of human phosphoglycerate mutase. Wang, Y., Cheng, Z., Liu, L., Wei, Z., Wan, M., Gong, W. Acta Crystallogr. D Biol. Crystallogr. (2004) [Pubmed]
  21. Manifesting heterozygotes in a Japanese family with a novel mutation in the muscle-specific phosphoglycerate mutase (PGAM-M) gene. Hadjigeorgiou, G.M., Kawashima, N., Bruno, C., Andreu, A.L., Sue, C.M., Rigden, D.J., Kawashima, A., Shanske, S., DiMauro, S. Neuromuscul. Disord. (1999) [Pubmed]
  22. Muscle phosphoglycerate mutase deficiency with tubular aggregates: effect of dantrolene. Vissing, J., Schmalbruch, H., Haller, R.G., Clausen, T. Ann. Neurol. (1999) [Pubmed]
  23. Human erythrocyte bisphosphoglycerate mutase: inactivation by glycation in vivo and in vitro. Fujita, T., Suzuki, K., Tada, T., Yoshihara, Y., Hamaoka, R., Uchida, K., Matuo, Y., Sasaki, T., Hanafusa, T., Taniguchi, N. J. Biochem. (1998) [Pubmed]
  24. Phosphoglycerate mutase, 2,3-bisphosphoglycerate phosphatase, creatine kinase and enolase activity and isoenzymes in breast carcinoma. Durany, N., Joseph, J., Jimenez, O.M., Climent, F., Fernández, P.L., Rivera, F., Carreras, J. Br. J. Cancer (2000) [Pubmed]
  25. Myopathies due to enzyme deficiencies. Cornelio, F., Di Donato, S. J. Neurol. (1985) [Pubmed]
  26. Chamber-specific expression of human myocardial proteins detected by two-dimensional gel electrophoresis. Pleissner, K.P., Regitz-Zagrosek, V., Weise, C., Neuss, M., Krüdewagen, B., Söding, P., Buchner, K., Hucho, F., Hildebrandt, A., Fleck, E. Electrophoresis (1995) [Pubmed]
  27. A phosphoglycerate mutase brain isoform (PGAM 1) pseudogene is localized within the human Menkes disease gene (ATP7 A). Dierick, H.A., Mercer, J.F., Glover, T.W. Gene (1997) [Pubmed]
  28. Mouse phosphoglycerate mutase M and B isozymes: cDNA cloning, enzyme activity assay and mapping. Zhang, J., Yu, L., Fu, Q., Gao, J., Xie, Y., Chen, J., Zhang, P., Liu, Q., Zhao, S. Gene (2001) [Pubmed]
  29. The identification of multiple molecular forms of human red cell phosphoglycerate mutase and 2,3-bisphosphoglycerate synthase by isoelectric focusing. Hass, L.F., Miller, K.B. J. Biol. Chem. (1978) [Pubmed]
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