Browsing Pathways

L-2-Hydroxyglutaric Aciduria (D-2-Hydroxyglutaric Aciduria ) is an autosomal recessive disease caused by a mutation in the L2HGDH gene which codes for L-2-Hydroxygluarate dehydrogenase. A deficiency in this enzyme results in accumulation of L-2-Hydroxyglutaric acid in plasma, spinal fluid, and urine; and L-lysine in plasma and spinal fluid. Symptoms, which present at birth, include ataxia, hypotonia, mental retardation, and seizures. Premature death often results. D-2-Hydroxyglutaric Aciduria is an autosomal recessive disease caused by a mutation in the D2HGDH gene which does for D-2-Hydroxygluarate dehydrogenase. A deficiency in this enzyme results in accumulation of D-2-Hydroxyglutaric acid in plasma, spinal fluid, and urine; oxoglutaric acid in urine; and gabba-aminobutyric acid in spinal fluid. Symptoms, which present at birth, include ataxia, hypotonia, mental retardation, and seizures. Premature death often results.

2-Ketoglutarate dehydrogenase complex deficiency, also known as alpha-ketoglutarate dehydrogenase deficiency or oxoglutaric aciduria, is an autosomal recessive disorder of the Krebs cycle caused by a defective oxoglutarate dehydrogenase complex (OGDC). OGDC catalyzes the conversion of 2-ketoglutarate into succinyl-CoA. This disorder is characterized by a large accumulation of 2-ketoglutarate in the urine. Symptoms of the disorder include opisthotonus, ataxia, developmental delay, and seizures.

2-Ketoglutarate dehydrogenase complex deficiency, also known as alpha-ketoglutarate dehydrogenase deficiency or oxoglutaric aciduria, is an autosomal recessive disorder of the Krebs cycle caused by a defective oxoglutarate dehydrogenase complex (OGDC). OGDC catalyzes the conversion of 2-ketoglutarate into succinyl-CoA. This disorder is characterized by a large accumulation of 2-ketoglutarate in the urine. Symptoms of the disorder include opisthotonus, ataxia, developmental delay, and seizures.

2-Ketoglutarate dehydrogenase complex deficiency, also known as alpha-ketoglutarate dehydrogenase deficiency or oxoglutaric aciduria, is an autosomal recessive disorder of the Krebs cycle caused by a defective oxoglutarate dehydrogenase complex (OGDC). OGDC catalyzes the conversion of 2-ketoglutarate into succinyl-CoA. This disorder is characterized by a large accumulation of 2-ketoglutarate in the urine. Symptoms of the disorder include opisthotonus, ataxia, developmental delay, and seizures.

2-Methyl-3-hydroxybutyryl CoA dehydrogenase deficiency (Hydroxyl-CoA dehydrogenase deficiency; MHBD) is a rare inborn disease of metabolism caused by a mutation in the HSD17B10 gene which codes for 3-hydroxyacyl-CoA dehydrogenase type-2. A deficiency in this enzyme results in accumulation of L-lactic acid in blood, spinal fluid, and urine; 2-ethylhydracrylic acid, 2-methyl-3-hydroxybutyric acid, and tiglylglycine in urine. Symptoms include cerebal atrophy, motor and mental retardation, overactivity and behavior issues, seizures and progressive neurological defects leading to early death. Treatment includes a high carbohydrate and low protein diet.

2-Methyl-3-hydroxybutyryl CoA dehydrogenase deficiency (Hydroxyl-CoA dehydrogenase deficiency; MHBD) is a rare inborn disease of metabolism caused by a mutation in the HSD17B10 gene which codes for 3-hydroxyacyl-CoA dehydrogenase type-2. A deficiency in this enzyme results in accumulation of L-lactic acid in blood, spinal fluid, and urine; 2-ethylhydracrylic acid, 2-methyl-3-hydroxybutyric acid, and tiglylglycine in urine. Symptoms include cerebal atrophy, motor and mental retardation, overactivity and behavior issues, seizures and progressive neurological defects leading to early death. Treatment includes a high carbohydrate and low protein diet.

2-Methyl-3-hydroxybutyryl CoA dehydrogenase deficiency (Hydroxyl-CoA dehydrogenase deficiency; MHBD) is a rare inborn disease of metabolism caused by a mutation in the HSD17B10 gene which codes for 3-hydroxyacyl-CoA dehydrogenase type-2. A deficiency in this enzyme results in accumulation of L-lactic acid in blood, spinal fluid, and urine; 2-ethylhydracrylic acid, 2-methyl-3-hydroxybutyric acid, and tiglylglycine in urine. Symptoms include cerebal atrophy, motor and mental retardation, overactivity and behavior issues, seizures and progressive neurological defects leading to early death. Treatment includes a high carbohydrate and low protein diet.

2-O-α-Mannosyl-D-glycerate (MG; also named as Alpha-Mannosylglycerate) is an organic compound that will affect the osmosis in hyperthermophilic archaea and bacteria. In E.coli, 2-O-α-mannosyl-D-glycerate PTS permease (mngA) import MG into cell, and then phosphorylate MG to 2-O-(6-phospho-α-mannosyl)-D-glycerate by phosphocarrier protein HPr. 2-O-(6-phospho-α-mannosyl)-D-glycerate is converted to glyceric acid as well as mannose 6-phosphate by alpha-mannosidase mngB. Finally, glyceric acid is catalyzed to 2-Phospho-D-glyceric acid with ATP as energy source by Glycerate kinase 2. E.coli can't use MG as osmotic stress protection, but it can use MG as a carbon source.

2-oxoglutarate dehydrogenase complex is consisted of oxoglutarate decarboxylase, dihydrolipoyl succinyltransferase and dihydrolipoyl dehydrogenase), which is a rate-limiting enzyme of the citric acid cycle (TCA cycle) in prokaryote. The reaction that catalyzed by 2-oxoglutarate dehydrogenase complex can be generalized as 2-oxoglutarate + coenzyme A + NAD+ → succinyl-CoA + CO2 + NADH. During the OGDHC reaction cycle, 2-oxoglutarate is bound and decarboxylated by E1(o), a thiamin-diphosphate cofactor containing enzyme. The succinyl group is transferred to the lipoyl domain of E2(o) where it is carried to the active site and transferred to coenzyme A, forming succinyl-CoA. During this transfer the lipoyl group is reduced to dihydrolipoyl. The succinyl-CoA is released and the lipoyl domain of E2(o) is oxidized by E3 via transfer of protons to NAD, forming NADH and regenerating the lipoyl group back to lipoyllysine for another cycle. Under aerobic growth conditions the OGDHC not only catalyzes a key reaction in the TCA cycle, it also provides succinyl-CoA for methionine and lysine biosynthesis, the latter pathway also leading to peptidoglycan biosynthesis. The synthesis of the OGDHC is repressed by anaerobiosis and is also subject to glucose repression. It is induced by aerobic growth on acetate. (EcoCyc)

2-oxoglutarate dehydrogenase complex is consisted of oxoglutarate decarboxylase, dihydrolipoyl succinyltransferase and dihydrolipoyl dehydrogenase), which is a rate-limiting enzyme of the citric acid cycle (TCA cycle) in prokaryote. The reaction that catalyzed by 2-oxoglutarate dehydrogenase complex can be generalized as 2-oxoglutarate + coenzyme A + NAD+ → succinyl-CoA + CO2 + NADH. During the OGDHC reaction cycle, 2-oxoglutarate is bound and decarboxylated by E1(o), a thiamin-diphosphate cofactor containing enzyme. The succinyl group is transferred to the lipoyl domain of E2(o) where it is carried to the active site and transferred to coenzyme A, forming succinyl-CoA. During this transfer the lipoyl group is reduced to dihydrolipoyl. The succinyl-CoA is released and the lipoyl domain of E2(o) is oxidized by E3 via transfer of protons to NAD, forming NADH and regenerating the lipoyl group back to lipoyllysine for another cycle. Under aerobic growth conditions the OGDHC not only catalyzes a key reaction in the TCA cycle, it also provides succinyl-CoA for methionine and lysine biosynthesis, the latter pathway also leading to peptidoglycan biosynthesis. The synthesis of the OGDHC is repressed by anaerobiosis and is also subject to glucose repression. It is induced by aerobic growth on acetate. (EcoCyc)