Pathways > Trifunctional Protein Deficiency
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Trifunctional Protein Deficiency

Proteins (14)

ProteinSynonymsTaxonomy
Trifunctional enzyme subunit alpha, mitochondrial78 kDa gastrin-binding protein; Monolysocardiolipin acyltransferase; 2.3.1.-; TP-alphaHomo sapiens (human)
Glutaryl-CoA dehydrogenase, mitochondrialGCD; EC 1.3.8.6Homo sapiens (human)
Enoyl-CoA hydratase, mitochondrialEC 4.2.1.17; Enoyl-CoA hydratase 1; Short-chain enoyl-CoA hydratase; SCEHHomo sapiens (human)
Trifunctional enzyme subunit beta, mitochondrialTP-betaHomo sapiens (human)
Medium-chain specific acyl-CoA dehydrogenase, mitochondrialMCAD; EC 1.3.8.7; Medium chain acyl-CoA dehydrogenase; MCADHHomo sapiens (human)
Long-chain-fatty-acid--CoA ligase 1EC 6.2.1.3; Acyl-CoA synthetase 1; ACS1; Arachidonate--CoA ligase; 6.2.1.15; Long-chain acyl-CoA synthetase 1; LACS 1; Long-chain acyl-CoA synthetase 2; LACS 2; Long-chain fatty acid-CoA ligase 2; Palmitoyl-CoA ligase 1; Palmitoyl-CoA ligase 2; Phytanate-Homo sapiens (human)
Very long-chain specific acyl-CoA dehydrogenase, mitochondrialVLCAD; EC 1.3.8.9Homo sapiens (human)
Carnitine O-palmitoyltransferase 2, mitochondrialEC 2.3.1.21; Carnitine palmitoyltransferase II; CPT IIHomo sapiens (human)
Carnitine O-palmitoyltransferase 1, liver isoformCPT1-L; EC 2.3.1.21; Carnitine O-palmitoyltransferase I, liver isoform; CPT I; CPTI-L; Carnitine palmitoyltransferase 1AHomo sapiens (human)
Acetyl-CoA acetyltransferase, mitochondrialEC 2.3.1.9; Acetoacetyl-CoA thiolase; T2Homo sapiens (human)
Short-chain specific acyl-CoA dehydrogenase, mitochondrialSCAD; EC 1.3.8.1; Butyryl-CoA dehydrogenaseHomo sapiens (human)
Long-chain specific acyl-CoA dehydrogenase, mitochondrialLCAD; EC 1.3.8.8Homo sapiens (human)
3-ketoacyl-CoA thiolase, mitochondrialEC 2.3.1.16; Acetyl-CoA acetyltransferase; 2.3.1.9; Acetyl-CoA acyltransferase; Acyl-CoA hydrolase, mitochondrial; 3.1.2.-; 3.1.2.1; 3.1.2.2; Beta-ketothiolase; Mitochondrial 3-oxoacyl-CoA thiolase; T1Homo sapiens (human)
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrialSBCAD; EC 1.3.8.5; 2-methyl branched chain acyl-CoA dehydrogenase; 2-MEBCAD; 2-methylbutyryl-coenzyme A dehydrogenase; 2-methylbutyryl-CoA dehydrogenaseHomo sapiens (human)

Compounds (18)

CompoundDescription
hydronium ion
levocarnitine
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
NADH
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 monophosphateAdenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position.
WaterA clear, odorless, tasteless liquid that is essential for most animal and plant life and is an excellent solvent for many substances. The chemical formula is hydrogen oxide (H2O). (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
palmitic acidA common saturated fatty acid found in fats and waxes including olive oil, palm oil, and body lipids.
Coenzyme A
glutaryl-coenzyme A
Palmitoyl Coenzyme AA fatty acid coenzyme derivative which plays a key role in fatty acid oxidation and biosynthesis.
S-tetradecanoyl-coenzyme A
palmitoylcarnitineA long-chain fatty acid ester of carnitine which facilitates the transfer of long-chain fatty acids from cytoplasm into mitochondria during the oxidation of fatty acids.
lauroyl-coenzyme a
hexanoyl-coenzyme a
NADA coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)
acetyl coenzyme aAcetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent.
pyrophosphate
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