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・ Phosphoenolpyruvate carboxykinase (ATP)
・ Phosphoenolpyruvate carboxykinase (diphosphate)
・ Phosphoenolpyruvate carboxylase
・ Phosphoenolpyruvate mutase
・ Phosphoenolpyruvate phosphatase
・ Phosphoenolpyruvate—glycerone phosphotransferase
・ Phosphoenolpyruvate—protein phosphotransferase
・ Phosphoenolpyruvic acid
・ Phosphoenolpyruvic carboxykinase
・ Phosphoenolpyruvic carboxylase
・ Phosphoethanolamine N-methyltransferase
・ Phosphoethanolamine/phosphocholine phosphatase
・ Phosphofructokinase
・ Phosphofructokinase 1
・ Phosphofructokinase 2
Phosphofructokinase deficiency
・ Phosphoglucan, water dikinase
・ Phosphoglucokinase
・ Phosphoglucomutase
・ Phosphoglucomutase (glucose-cofactor)
・ Phosphoglucomutase 3
・ Phosphogluconate 2-dehydrogenase
・ Phosphogluconate dehydratase
・ Phosphogluconate dehydrogenase
・ Phosphogluconate dehydrogenase (decarboxylating)
・ Phosphoglucosamine mutase
・ Phosphoglycerate
・ Phosphoglycerate dehydrogenase
・ Phosphoglycerate kinase
・ Phosphoglycerate kinase (GTP)


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Phosphofructokinase deficiency : ウィキペディア英語版
Phosphofructokinase deficiency

Phosphofructokinase deficiency, also known as Glycogen storage disease type VII or Tarui's disease, is metabolic disorder with autosomal recessive inheritance.
It may affect humans as well as other mammals (especially dogs). In humans it is the least common type of glycogen storage disease. It was named after the Japanese Physician, Seiichiro Tarui (1927- ).
==Pathophysiology==
In this condition, a deficiency of the M subunit (PFKM) of the phosphofructokinase enzyme impairs the ability of cells such as erythrocytes and rhabdomyocytes (skeletal muscle cells) to use carbohydrates (such as glucose) for energy.
The mutation impairs the ability of phosphofructokinase to phosphorylate fructose-6-phosphate prior to its cleavage into glyceraldehyde-3-phosphate which enters the energy generation phase of glycolysis, effectively limiting energy production.
Unlike most other glycogen storage diseases, it directly affects glycolysis.

抄文引用元・出典: フリー百科事典『 ウィキペディア(Wikipedia)
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