Pyruvate Kinase: Difference between revisions
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Red blood cells, in a state of pyruvate kinase deficiency, rapidly become deficient in ATP and can undergo hemolysis.This is transmitted as an autosomal recessive trit. The severity of hemolysis is extremely variable such as a mild case to life-threatening neonatal anaemia requiring transfusions. Over one hundred eighty different mutations have been discovered in relation to this deficiency with most being autosomal recessive, but a few strands are autosomal dominant. The deficiency causes red blood cells to deform into echinocytes on peripheral blood smears. This causes the buildup of reaction intermediates which can also increase the level of 2,3-bisphosphoglycerate in the cells. This causes a rightward shift in the hemoglobin oxygen saturation curve, which means that there is a decreased oxygen affinity for the hemoglobin and earlier oxygen unloading than under normal conditions | Red blood cells, in a state of pyruvate kinase deficiency, rapidly become deficient in ATP and can undergo hemolysis.This is transmitted as an autosomal recessive trit. The severity of hemolysis is extremely variable such as a mild case to life-threatening neonatal anaemia requiring transfusions. Over one hundred eighty different mutations have been discovered in relation to this deficiency with most being autosomal recessive, but a few strands are autosomal dominant. The deficiency causes red blood cells to deform into echinocytes on peripheral blood smears. This causes the buildup of reaction intermediates which can also increase the level of 2,3-bisphosphoglycerate in the cells. This causes a rightward shift in the hemoglobin oxygen saturation curve, which means that there is a decreased oxygen affinity for the hemoglobin and earlier oxygen unloading than under normal conditions | ||
<ref>PMID:17360088</ref>. | <ref>PMID:17360088</ref>. | ||
==3D structures of pyruvate kinase== | |||
===Pyruvate kinase=== | |||
[[3hqn]], [[3e0w]], [[1pkl]] – LmPyK – Leishmania mexicana<br /> | |||
[[3khd]] – PyK – Plasmodium falciparum<br /> | |||
[[3gg8]], [[3eoe]] – PyK – Toxoplasma gondii<br /> | |||
[[3g2g]] - hPyK M2 (mutant) – human<br /> | |||
[[3bjf]], [[3bjt]], [[1t5a]], [[1zjh]] - hPyK M2<br /> | |||
[[2vgb]] – hPyK<br /> | |||
[[2vgf]], [[2vgg]], [[2vgi]] - hPyK (mutant) <br /> | |||
[[3ma8]] – PyK – Cryptosporidium parvum<br /> | |||
[[2e28]] - PyK (mutant) – Geobacillus stearothermophilus<br /> | |||
[[1f3w]] – rPyK – rabbit<br /> | |||
[[1f3x]] - rPyK (mutant) <br /> | |||
[[1e0t]], [[1e0u]] - EcPyK (mutant) – Eschericia coli<br /> | |||
[[1pky]] – EcPyK<br /> | |||
[[1pkm]], [[1pyk]] – PyK – cat | |||
===Pyruvate kinase binary complex=== | |||
[[3qv9]] – PyK + Ponceau S – Trypanosoma cruzei<br /> | |||
[[3qv6]] – LmPyK + acid blue 80<br /> | |||
[[3e0v]] – LmPyK + sulfate<br /> | |||
[[3hqq]] - LmPyK + fructose bisphosphate<br /> | |||
[[3qv8]] – LmPyK + benzothiazole<br /> | |||
[[3pp7]] – LmPyK + suramin<br /> | |||
[[3is4]], [[3ktx]] – LmPyK + pyrenetetrasulfonic acid | |||
===Pyruvate kinase ternary complex=== | |||
[[3hqo]] – LmPyK + ATP + oxalate<br /> | |||
[[3hqp]] - LmPyK + ATP + oxalate + fructose bisphosphate<br /> | |||
[[3qv7]] – LmPyK + acid blue 25 + Ponceau S<br /> | |||
[[3n25]] – rPyK + proline + Mn + pyruvate<br /> | |||
[[2g50]] - rPyK + alanine + Mn + pyruvate<br /> | |||
[[1pkn]] - rPyK + Mn + pyruvate<br /> | |||
[[1aqf]] – rPyK + Mg + phospholactate<br /> | |||
[[1a49]], [[1a5u]] - rPyK + ATP + oxalate<br /> | |||
[[3me3]] – hPyK M2 + aniline derivative + fructose bisphosphate<br /> | |||
[[3h6o]] - hPyK M2 + pyridazine derivative + fructose bisphosphate<br /> | |||
[[3gqy]], [[3gr4]] - hPyK M2 + piperazine derivative + fructose bisphosphate<br /> | |||
[[1a3w]] – yPyK + Mn + phosphoglycolic acid + fructose bisphosphate – yeast<br /> | |||
[[1a3x]] - yPyK + Mn + phosphoglycolic acid | |||
==Additional Resources== | ==Additional Resources== | ||
