Pathways > Krebs cycle disorders
Page last updated: 2024-10-06

Krebs cycle disorders

Proteins (12)

ProteinSynonymsTaxonomy
Alanine aminotransferase 1ALT1; EC 2.6.1.2; Glutamate pyruvate transaminase 1; GPT 1; Glutamic--alanine transaminase 1; Glutamic--pyruvic transaminase 1Homo sapiens (human)
L-lactate dehydrogenase A chainLDH-A; EC 1.1.1.27; Cell proliferation-inducing gene 19 protein; LDH muscle subunit; LDH-M; Renal carcinoma antigen NY-REN-59Homo sapiens (human)
Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrialEC 6.2.1.5; ATP-specific succinyl-CoA synthetase subunit beta; A-SCS; Succinyl-CoA synthetase beta-A chain; SCS-betaAHomo sapiens (human)
L-lactate dehydrogenase B chainLDH-B; EC 1.1.1.27; LDH heart subunit; LDH-H; Renal carcinoma antigen NY-REN-46Homo sapiens (human)
Multifunctional 2-oxoglutarate metabolism enzyme2-hydroxy-3-oxoadipate synthase; HOA synthase; HOAS; 2.2.1.5; 2-oxoglutarate carboxy-lyase; 2-oxoglutarate decarboxylase; Alpha-ketoglutarate decarboxylase; KG decarboxylase; KGD; 4.1.1.71; Alpha-ketoglutarate-glyoxylate carboligaseMycobacterium sp. MCS
L-lactate dehydrogenase C chainLDH-C; EC 1.1.1.27; Cancer/testis antigen 32; CT32; LDH testis subunit; LDH-XHomo sapiens (human)
Pyruvate dehydrogenase protein X component, mitochondrialDihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex; E3-binding protein; E3BP; Lipoyl-containing pyruvate dehydrogenase complex component X; proXHomo sapiens (human)
Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrialEC 1.2.4.2; Dehydrogenase E1 and transketolase domain-containing protein 1Homo sapiens (human)
Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrialEC 6.2.1.4; EC 6.2.1.5; Succinyl-CoA synthetase subunit alpha; SCS-alphaHomo sapiens (human)
Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrialEC 2.3.1.61; 2-oxoglutarate dehydrogenase complex component E2; OGDC-E2; Dihydrolipoamide succinyltransferase component of 2-oxoglutarate dehydrogenase complex; E2KHomo sapiens (human)
Succinate--CoA ligase [GDP-forming] subunit beta, mitochondrialEC 6.2.1.4; GTP-specific succinyl-CoA synthetase subunit beta; G-SCS; GTPSCS; Succinyl-CoA synthetase beta-G chain; SCS-betaGHomo sapiens (human)
Mitochondrial pyruvate carrier 1Brain protein 44-like proteinHomo sapiens (human)

Compounds (11)

CompoundDescription
Succinate
fumaric acidsee also record for ferrous fumarate; use FUMARATES for general fumaric acid esters
Adenosine TriphosphateAn adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.
adenosine diphosphateAdenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.
guanosine diphosphateA guanine nucleotide containing two phosphate groups esterified to the sugar moiety.
guanosine triphosphateGuanosine 5'-(tetrahydrogen triphosphate). A guanine nucleotide containing three phosphate groups esterified to the sugar moiety.
Pyruvic AcidAn intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed)
alpha-Ketoglutarate
methylmalonic acidA malonic acid derivative which is a vital intermediate in the metabolism of fat and protein. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This metabolic disease is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA.
alanineA non-essential amino acid that occurs in high levels in its free state in plasma. It is produced from pyruvate by transamination. It is involved in sugar and acid metabolism, increases IMMUNITY, and provides energy for muscle tissue, BRAIN, and the CENTRAL NERVOUS SYSTEM.
succinyl-coenzyme a
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