Phosphoglycerate Mutase: Difference between revisions
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== Background == | == Background == | ||
Glycolysis is a 10-step process that invests energy in the initial stages only to recover greater amounts of energy in the final steps. Every step in this metabolic pathway is essential to the ultimate production of energy. Every step is catalyzed by one or more enzymes that enhance the rate of the given reaction. Phosphoglycerate mutase is the specific homotetramer enzyme that catalyzes step 8 of glycolysis transfering the phosphate from 3-phosphoglyceric acid to the second carbon to form 2-phosphoglyceric acid, having the Protein Data Bank ID [[1qhf]]<ref>PMID:10531478</ref>. Phosphoglycerate mutase (PGM) is found in organisms from yeast to humans because it plays a significant role in glycolysis, which is a highly conserved process across many taxa. A deficiency of this enzyme causes CNS symptoms, muscle weakness, cramps and fatigue with exercise. <ref>http://disability.ucdavis.edu/disease_deatails.php?id=45</ref> | Glycolysis is a 10-step process that invests energy in the initial stages only to recover greater amounts of energy in the final steps. Every step in this metabolic pathway is essential to the ultimate production of energy. Every step is catalyzed by one or more enzymes that enhance the rate of the given reaction. Phosphoglycerate mutase is the specific homotetramer enzyme that catalyzes step 8 of glycolysis transfering the phosphate from 3-phosphoglyceric acid to the second carbon to form 2-phosphoglyceric acid, having the Protein Data Bank ID [[1qhf]]<ref>PMID:10531478</ref>. '''Phosphoglycerate mutase''' (PGM) is found in organisms from yeast to humans because it plays a significant role in glycolysis, which is a highly conserved process across many taxa. A deficiency of this enzyme causes CNS symptoms, muscle weakness, cramps and fatigue with exercise. <ref>http://disability.ucdavis.edu/disease_deatails.php?id=45</ref> | ||
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== Phosphoglycerate Mutase Deficiency == | == Phosphoglycerate Mutase Deficiency == | ||
When phosphoglycerate mutase has a genetic defect, it results in a muscle disease that interferes with the processing of carbohydrates. The onset can occur anywhere from childhood to adulthood. The inheritance pattern is autosomal recessive. <ref>http://www.mda.org/disease/pgam.html</ref> Phosphoglycerate mutase deficicncy patients may experience CNS symptoms such as mental retardation and seizures. Certain individuals may experience a purely myopathic syndrome with progressive proximal muscle weakness and incidents of myoglobinuria, exercise intolerance, may become easy fatigued with cramps and urine discoloration. Diagnosing this deficiency can be done with Laboratory tests that demonstrate and increased serum CK level. Or there are diagnostic tests available that test for the absence of the enzyme. Also, muscle pathology of this deficiency shows subsarcolemmal glycogen ± tubular combinations. <ref>http://disability.ucdavis.edu/disease_deatails.php?id=45</ref> | When phosphoglycerate mutase has a genetic defect, it results in a muscle disease that interferes with the processing of carbohydrates. The onset can occur anywhere from childhood to adulthood. The inheritance pattern is autosomal recessive. <ref>http://www.mda.org/disease/pgam.html</ref> Phosphoglycerate mutase deficicncy patients may experience CNS symptoms such as mental retardation and seizures. Certain individuals may experience a purely myopathic syndrome with progressive proximal muscle weakness and incidents of myoglobinuria, exercise intolerance, may become easy fatigued with cramps and urine discoloration. Diagnosing this deficiency can be done with Laboratory tests that demonstrate and increased serum CK level. Or there are diagnostic tests available that test for the absence of the enzyme. Also, muscle pathology of this deficiency shows subsarcolemmal glycogen ± tubular combinations. <ref>http://disability.ucdavis.edu/disease_deatails.php?id=45</ref> | ||
==3D structures of phosphoglycerate mutase== | |||
===Phosphoglycerate mutase=== | |||
[[3nvl]] – PGM - Trypanosoma brucei <br /> | |||
[[3o0t]], [[3mxo]] – hPGM5 residues 90-289 – human<br /> | |||
[[1yfk]], [[1yjx]] – hPGM1<br /> | |||
[[3ezn]] – BpPGM – Burkholderia pseudomallei<br /> | |||
[[3d8h]] – PGM – Cryptosporidium parvum<br /> | |||
[[2ify]] – PGM – Bacillus anthracis<br /> | |||
[[1v7q]], [[1v37]] – PGM – Thermus thermophilus<br /> | |||
[[1xq9]] - PfPGM – Plasmodium falciparum<br /> | |||
[[3kkk]] - PfPGM (mutant) <br /> | |||
[[1rii]] – PGM – Mycobacteriumtuberculosis<br /> | |||
[[1e58]] – EcPGM – Escherichia coli<br /> | |||
[[1fzt]] – PGM – Fission yeast – NMR<br /> | |||
[[5pgm]], [[4pgm]], [[3pgm]] – yPGM – yeast<br /> | |||
[[3idd]] – PGM – Thermoplasma acidophilum<br /> | |||
[[2zkt]] – PGM – Pyrococcus horikoshii | |||
===Phosphoglycerate mutase binary complexes=== | |||
[[3lnt]] – BpPGM + malonic acid <br /> | |||
[[3gw8 – BpPGM + glycerol + VO4<BR /> | |||
[[3gp5]] - BpPGM + PGA + VO4<BR /> | |||
[[3fdz]] - BpPGM + PGA + di-PGA<br /> | |||
[[3gp3]] – BpPGM + phosphoserine<br /> | |||
[[3igy]], [[3igz]] – PGM + Co + PGA – Leishmania mexicana<br /> | |||
[[1o98]], [[1eqj]], [[1ejj]] – BsPGM + PGA – Bacillus stearothermophilus<br /> | |||
[[1o99]] - BsPGM (mutant) + PGA<br /> | |||
[[1e59]] – EcPGM + VO4<BR /> | |||
[[1qhf]] – yPGM + PGA <br /> | |||
[[1bq3]] – yPGM + inositol hexakisphosphate<br /> | |||
[[1bq4]] – yPGM + benzene hexacarboxylate | |||
==Additional Resources== | ==Additional Resources== | ||
For additional information, see: [[Carbohydrate Metabolism]] | For additional information, see: [[Carbohydrate Metabolism]] | ||