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PathWhiz ID Pathway Meta Data

PW121850

Pw121850 View Pathway
disease

D-Glyceric Acidura

Mus musculus
D-Glyceric aciduria is an extremely rare inherited inborn error of metabolism (IEM) of serine and fructose metabolism. It is an autosomal recessive disorder that is caused by a defect in the D-glycerate kinase (GLYCTK) gene. GLYCTK codes for D-glycerate kinase, an enzyme that is responsible for phosphorylating D-glyceric acid into phosphoglycerate. D-Glycerate kinase is an enzyme that participates in 3 metabolic pathways: (1) serine/glycine/threonine metabolism, (2) glycerolipid metabolism, and (3) glyoxylate-dicarboxylate metabolism (which is a minor pathway in fructose metabolism). Defects in the enzyme will lead to accumulations of D-glyceric acid in tissues and biofluids. D-Glyceric aciduria was first described in 1974 and is characterized by elevated levels of D-glyceric acid in the urine. Clinical symptoms of D-glyceric aciduria are highly variable. Some patients have neurological symptoms, with severe mental retardation, seizures, microcephaly, and sometimes early death, whereas others have a mild phenotype with only mild speech delay or even normal development.

PW127272

Pw127272 View Pathway
disease

D-Glyceric Aciduria

Homo sapiens
D-Glyceric aciduria is an extremely rare inherited inborn error of metabolism (IEM) of serine and fructose metabolism. It is an autosomal recessive disorder that is caused by a defect in the D-glycerate kinase (GLYCTK) gene. GLYCTK codes for D-glycerate kinase, an enzyme that is responsible for phosphorylating D-glyceric acid into phosphoglycerate. D-Glycerate kinase is an enzyme that participates in 3 metabolic pathways: (1) serine/glycine/threonine metabolism, (2) glycerolipid metabolism, and (3) glyoxylate-dicarboxylate metabolism (which is a minor pathway in fructose metabolism). Defects in the enzyme will lead to accumulations of D-glyceric acid in tissues and biofluids. D-Glyceric aciduria was first described in 1974 and is characterized by elevated levels of D-glyceric acid in the urine. Clinical symptoms of D-glyceric aciduria are highly variable. Some patients have neurological symptoms, with severe mental retardation, seizures, microcephaly, and sometimes early death, whereas others have a mild phenotype with only mild speech delay or even normal development.

PW145518

Pw145518 View Pathway
drug action

D-Methionine Drug Metabolism Action Pathway

Homo sapiens

PW012890

Pw012890 View Pathway
metabolic

D-myo-Inositol (1,4,5)-Trisphosphate Biosynthesis

Arabidopsis thaliana
D-myo-inositol (1,4,5)-trisphosphate biosynthesis is a pathway that occurs in the cytosol by which myo-inositol becomes D-myo-inositol (1,4,5)-trisphosphate (IP3), a secondary messenger molecule used in signal transduction and lipid signaling in biological cells . This pathway consists of a few cell membrane-associated enzymes (coloured dark green in the image): phosphatidylinositol 4-phosphate 5-kinase and phosphoinositide phospholipase C. First, phosphatidylinositol synthase uses a a CDP-diacylglycerol to catalyze a reaction whereby myo-inositol is converted into an an L-1-phosphatidyl-inositol. It requires magnesium or manganese ions as cofactors. Second, phosphatidylinositol 4-kinase uses ATP to catalyze the conversion of an L-1-phosphatidyl-inositol to a 1-phosphatidyl-1D-myo-inositol 4-phosphate. Third, phosphatidylinositol 4-phosphate 5-kinase uses ATP to catalyze the conversion of a 1-phosphatidyl-1D-myo-inositol 4-phosphate to a 1-phosphatidyl-1D-myo-inositol 4,5-bisphosphate. Alternatively, 1-phosphatidyl-1D-myo-inositol 4,5-bisphosphate is predicted to be synthesized by a still undiscovered phosphatidylinositol-5-phosphate 4-kinase from ATP and a 1-phosphatidyl-1D-myo-inositol 5-phosphate. Finally, phosphoinositide phospholipase C uses water to catalyze the conversion of a 1-phosphatidyl-1D-myo-inositol 4,5-bisphosphate to D-myo-inositol (1,4,5)-trisphosphate, producing 1,2-diacyl-sn-glycerol and a proton as byproducts. Phosphoinositide phospholipase C requires calcium as a cofactor.

PW126912

Pw126912 View Pathway
metabolic

d-psicose metabolism

Homo sapiens

PW342424

Pw342424 View Pathway
metabolic

D-Serine Degradation

Fusobacterium periodonticum 1_1_41FAA
The degradation of D-serine begins with the transport of D-serine into the cytosol through a cycA. Once in the cytosol D-serine reacts with ammonia-lyase resulting in the release of a hydrogen ion, water and a 2-aminoprop-2-enoate. This compound in turn reacts spontaneously to produces 2-iminipropanoate. This compound in turn reacts with water and hydrogen ion spontaneously resulting in the release of ammonium and apyruvate.

PW342400

Pw342400 View Pathway
metabolic

D-Serine Degradation

Capnocytophaga sputigena ATCC 33612
The degradation of D-serine begins with the transport of D-serine into the cytosol through a cycA. Once in the cytosol D-serine reacts with ammonia-lyase resulting in the release of a hydrogen ion, water and a 2-aminoprop-2-enoate. This compound in turn reacts spontaneously to produces 2-iminipropanoate. This compound in turn reacts with water and hydrogen ion spontaneously resulting in the release of ammonium and apyruvate.

PW342378

Pw342378 View Pathway
metabolic

D-Serine Degradation

Parabacteroides johnsonii DSM 18315
The degradation of D-serine begins with the transport of D-serine into the cytosol through a cycA. Once in the cytosol D-serine reacts with ammonia-lyase resulting in the release of a hydrogen ion, water and a 2-aminoprop-2-enoate. This compound in turn reacts spontaneously to produces 2-iminipropanoate. This compound in turn reacts with water and hydrogen ion spontaneously resulting in the release of ammonium and apyruvate.

PW358952

Pw358952 View Pathway
metabolic

D-Serine Degradation

Escherichia coli O157:H7 str. TW14359
The degradation of D-serine begins with the transport of D-serine into the cytosol through a cycA. Once in the cytosol D-serine reacts with ammonia-lyase resulting in the release of a hydrogen ion, water and a 2-aminoprop-2-enoate. This compound in turn reacts spontaneously to produces 2-iminipropanoate. This compound in turn reacts with water and hydrogen ion spontaneously resulting in the release of ammonium and apyruvate.

PW358957

Pw358957 View Pathway
metabolic

D-Serine Degradation

Escherichia coli O55:H7 str. CB9615
The degradation of D-serine begins with the transport of D-serine into the cytosol through a cycA. Once in the cytosol D-serine reacts with ammonia-lyase resulting in the release of a hydrogen ion, water and a 2-aminoprop-2-enoate. This compound in turn reacts spontaneously to produces 2-iminipropanoate. This compound in turn reacts with water and hydrogen ion spontaneously resulting in the release of ammonium and apyruvate.