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Pathways

Showing 11 - 20 of 61345 pathways
SMPDB ID Pathway Chemical Compounds Proteins

SMP00774

Pw000751 View Pathway
metabolic

2,3-dihydroxybenzoate biosynthesis

Escherichia coli
2,3-dihydroxybenzoate is synthesized from chorismate via isochorismate and 2,3-dihydroxy-2,3-dihydrobenzoate. Chorismate is a key intermediate and branch point in the biosynthesis of many aromatic compounds. The biosynthesis of 2,3-dihydroxybenzoate from chorismate is catalyzed by three enzymes EntC, EntB, and EntA. EntC catalyzes the conversion of chorismate to isochorismate. The N-terminal isochorismate lyase domain of EntB hydrolyzes the pyruvate group of isochorismate to produce 2,3-dihydro-2,3-dihydroxybenzoate. The conversion of this latter compound to 2,3-dihydroxybenzoate is catalyzed by the EntA dehydrogenase.

SMP00719

Pw000696 View Pathway
disease

2-aminoadipic 2-oxoadipic aciduria

Homo sapiens
Mutations in DHTKD1 (dehydrogenase E1 and transketolase domain-containing protein 1) have been shown to cause human 2-aminoadipic and 2-oxoadipic aciduria via impaired turnover of decarboxylation 2-oxoadipate to glutaryl-CoA, which is the last step in the lysine degradation pathway (PMID: 23141293 ).It is a metabolic disorder characterized by increased levels of 2-oxoadipate and 2-hydroxyadipate in the urine. Patients can have mild to severe intellectual disability, muscular hypotonia, developmental delay, ataxia, and epilepsy. Most cases are asymptomatic.

SMP00136

Pw000212 View Pathway
disease

2-Hydroxyglutric Aciduria (D And L Form)

Homo sapiens
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.

SMP00549

Pw000525 View Pathway
disease

2-ketoglutarate dehydrogenase complex deficiency

Homo sapiens
2-Ketoglutarate dehydrogenase complex deficiency is a rare autosomal recessive disease. 2-ketoglutarate dehydrogenase is an enzyme of the Krebs cycle that catalyzes the oxidation of alpha-ketoglutarate to succinyl CoA. The deficiency of 2-Ketoglutarate dehydrogenase complex results in the disorder of Krebs cycle with accumulation of succinyl CoA. The primary manifestations include developmental delay, ataxia, opisthotonus, seizure and other neurological symptoms.

SMP00137

Pw000061 View Pathway
disease

2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency

Homo sapiens
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.

SMP02108

Pw002096 View Pathway
metabolic

2-O-alpha-mannosyl-D-glycerate degradation

Escherichia coli
2-O-α-Mannosyl-D-glycerate (MG) is an osmolyte utilized by hyperthermophilic archaea and bacteria. E. coli is able to utilize MG as a carbon source but not as protection against osmotic stress. MG utilization is controlled by the divergently transcribed mngR gene and mngAB operon. MngR acts as a repressor of the expression both mngR and mngAB. MngA is the EII of a PEP-dependent sugar phosphotransferase system responsible for the uptake and phosphorylation of MG to 2-O-(6-phospho-α-mannosyl)-D-glycerate, which is subsequently converted to mannose-6-phosphate and glycerate by the α-mannosidase MngB. Glycerate can be converted to 2-phosphoglycerate by glycerate kinase I encoded by the garK gene which is also induced when cells are grown in MG. (EcoCyc)

SMP02120

Pw002108 View Pathway
metabolic

2-oxoglutarate decarboxylation to succinyl-CoA

Escherichia coli
The pathway illustrated here shows the reactions catalyzed by the 2-oxoglutarate dehydrogenase complex, a key, rate-limiting enzyme of the TCA cycle I (prokaryotic). These reactions can be summarized by the general reaction 2-oxoglutarate + coenzyme A + NAD+ → succinyl-CoA + CO2 + NADH which is the form commonly found in the TCA cycle. 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)

SMP01904

Pw001890 View Pathway
metabolic

2-Oxopent-4-enoate metabolism

Escherichia coli
The pathway starts with trans-cinnamate interacting with a hydrogen ion, an oxygen molecule, and a NADH through a cinnamate dioxygenase resulting in a NAD and a cis-3-(3-Carboxyethenyl)-3,5-cyclohexadiene-1,2-diol which then interact together through a 2,3-dihydroxy-2,3-dihydrophenylpropionate dehydrogenase resulting in the release of a hydrogen ion, an NADH molecule and a 2,3 dihydroxy-trans-cinnamate. The second way by which the 2,3 dihydroxy-trans-cinnamate is acquired is through a 3-hydroxy-trans-cinnamate interacting with a hydrogen ion, a NADH and an oxygen molecule through a 3-(3-hydroxyphenyl)propionate 2-hydroxylase resulting in the release of a NAD molecule, a water molecule and a 2,3-dihydroxy-trans-cinnamate. The compound 2,3 dihydroxy-trans-cinnamate then interacts with an oxygen molecule through a 2,3-dihydroxyphenylpropionate 1,2-dioxygenase resulting in a hydrogen ion and a 2-hydroxy-6-oxonona-2,4,7-triene-1,9-dioate. The latter compound then interacts with a water molecule through a 2-hydroxy-6-oxononatrienedioate hydrolase resulting in a release of a hydrogen ion, a fumarate molecule and (2Z)-2-hydroxypenta-2,4-dienoate. The latter compound reacts spontaneously to isomerize into a 2-oxopent-4-enoate. This compound is then hydrated through a 2-oxopent-4-enoate hydratase resulting in a 4-hydroxy-2-oxopentanoate. This compound then interacts with a 4-hydroxy-2-ketovalerate aldolase resulting in the release of a pyruvate, and an acetaldehyde. The acetaldehyde then interacts with a coenzyme A and a NAD molecule through a acetaldehyde dehydrogenase resulting in a hydrogen ion, a NADH and an acetyl-coa which can be incorporated into the TCA cycle

SMP02049

Pw002035 View Pathway
metabolic

2-Oxopent-4-enoate metabolism 2

Escherichia coli
The pathway starts with trans-cinnamate interacting with a hydrogen ion, an oxygen molecule, and a NADH through a cinnamate dioxygenase resulting in a NAD and a Cis-3-(3-carboxyethyl)-3,5-cyclohexadiene-1,2-diol which then interact together through a 2,3-dihydroxy-2,3-dihydrophenylpropionate dehydrogenase resulting in the release of a hydrogen ion, an NADH molecule and a 2,3 dihydroxy-trans-cinnamate. The second way by which the 2,3 dihydroxy-trans-cinnamate is acquired is through a 3-hydroxy-trans-cinnamate interacting with a hydrogen ion, a NADH and an oxygen molecule through a 3-(3-hydroxyphenyl)propionate 2-hydroxylase resulting in the release of a NAD molecule, a water molecule and a 2,3-dihydroxy-trans-cinnamate. The compound 2,3 dihydroxy-trans-cinnamate then interacts with an oxygen molecule through a 2,3-dihydroxyphenylpropionate 1,2-dioxygenase resulting in a hydrogen ion and a 2-hydroxy-6-oxonona-2,4,7-triene-1,9-dioate. The latter compound then interacts with a water molecule through a 2-hydroxy-6-oxononatrienedioate hydrolase resulting in a release of a hydrogen ion, a fumarate molecule and (2Z)-2-hydroxypenta-2,4-dienoate. The latter compound reacts spontaneously to isomerize into a 2-oxopent-4-enoate. This compound is then hydrated through a 2-oxopent-4-enoate hydratase resulting in a 4-hydroxy-2-oxopentanoate. This compound then interacts with a 4-hydroxy-2-ketovalerate aldolase resulting in the release of a pyruvate, and an acetaldehyde. The acetaldehyde then interacts with a coenzyme A and a NAD molecule through a acetaldehyde dehydrogenase resulting in a hydrogen ion, a NADH and an acetyl-coa which can be incorporated into the TCA cycle

SMP00576

Pw000552 View Pathway
disease

21-hydroxylase deficiency (CYP21)

Homo sapiens
Congenital adrenal hyperplasia (CAH) refers to any of several autosomal recessive diseases resulting from mutations of genes for enzymes mediating the biochemical steps of production of cortisol from cholesterol by the adrenal glands (steroidogenesis). 21-hydroxylase deficiency is an inherited disorder that affects the adrenal glands. The adrenal glands are located on top of the kidneys and produce a variety of hormones that regulate many essential functions in the body. In people with 21-hydroxylase deficiency, the adrenal glands produce excess androgens, which are male sex hormones. There are three types of 21-hydroxylase deficiency. Two types are classic forms, known as the salt-wasting and simple virilizing types. The third type is called the non-classic type. The salt-wasting type is the most severe, the simple virilizing type is less severe, and the non-classic type is the least severe form.
Showing 11 - 20 of 61345 pathways