Page last updated: 2024-10-06

Peroxisomal lipid metabolism

Proteins (20)

ProteinSynonymsTaxonomy
Very long-chain acyl-CoA synthetaseVLACS; VLCS; EC 6.2.1.-; Arachidonate--CoA ligase; 6.2.1.15; Fatty acid transport protein 2; FATP-2; Fatty-acid-coenzyme A ligase, very long-chain 1; Long-chain-fatty-acid--CoA ligase; 6.2.1.3; Phytanate--CoA ligase; 6.2.1.24; Solute carrier family 27 memHomo sapiens (human)
Peroxisomal carnitine O-octanoyltransferaseCOT; EC 2.3.1.137Homo sapiens (human)
Alpha-methylacyl-CoA racemase EC 5.1.99.4; 2-methylacyl-CoA racemaseHomo sapiens (human)
Hydroxyacid oxidase 2HAOX2; EC 1.1.3.15; (S)-2-hydroxy-acid oxidase, peroxisomal; Cell growth-inhibiting gene 16 protein; Long chain alpha-hydroxy acid oxidase; Long-chain L-2-hydroxy acid oxidaseHomo sapiens (human)
Carnitine O-acetyltransferaseCarnitine acetylase; EC 2.3.1.137; EC 2.3.1.7; Carnitine acetyltransferase; CAT; CrATHomo sapiens (human)
Phytanoyl-CoA dioxygenase, peroxisomalEC 1.14.11.18; Phytanic acid oxidase; Phytanoyl-CoA alpha-hydroxylase; PhyHHomo sapiens (human)
Peroxisomal bifunctional enzymePBE; PBFE; L-bifunctional protein; LBP; Multifunctional enzyme 1; MFE1Homo sapiens (human)
Peroxisomal multifunctional enzyme type 2MFE-2; 17-beta-hydroxysteroid dehydrogenase 4; 17-beta-HSD 4; D-bifunctional protein; DBP; Multifunctional protein 2; MFP-2; Short chain dehydrogenase/reductase family 8C member 1Homo sapiens (human)
Peroxisomal acyl-coenzyme A oxidase 2EC 1.17.99.3; 3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanoyl-CoA 24-hydroxylase; 3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanoyl-CoA oxidase; Trihydroxycoprostanoyl-CoA oxidase; THCA-CoA oxidase; THCCoxHomo sapiens (human)
Peroxisomal acyl-coenzyme A oxidase 3EC 1.3.3.6; Branched-chain acyl-CoA oxidase; BRCACox; Pristanoyl-CoA oxidaseHomo sapiens (human)
Acyl-coenzyme A thioesterase 8Acyl-CoA thioesterase 8; EC 3.1.2.1; EC 3.1.2.11; EC 3.1.2.2; EC 3.1.2.3; EC 3.1.2.5; Choloyl-coenzyme A thioesterase; 3.1.2.27; HIV-Nef-associated acyl-CoA thioesterase; Peroxisomal acyl-CoA thioesterase 2; PTE-2; Peroxisomal acyl-coenzyme A thioester hyHomo sapiens (human)
ATP-binding cassette sub-family D member 1EC 7.6.2.4; Adrenoleukodystrophy protein; ALDPHomo sapiens (human)
Nucleoside diphosphate-linked moiety X motif 19Nudix motif 19; EC 3.6.1.-Homo sapiens (human)
Peroxisomal membrane protein PMP3434 kDa peroxisomal membrane protein; Solute carrier family 25 member 17Homo sapiens (human)
3-ketoacyl-CoA thiolase, peroxisomalEC 2.3.1.16; Acetyl-CoA C-myristoyltransferase; 2.3.1.155; Acetyl-CoA acyltransferase; 2.3.1.9; Beta-ketothiolase; Peroxisomal 3-oxoacyl-CoA thiolaseHomo sapiens (human)
Peroxisomal coenzyme A diphosphatase NUDT7EC 3.6.1.-; Nucleoside diphosphate-linked moiety X motif 7; Nudix motif 7Homo sapiens (human)
Peroxisomal trans-2-enoyl-CoA reductaseTERP; EC 1.3.1.38; 2,4-dienoyl-CoA reductase-related protein; DCR-RP; HPDHase; Short chain dehydrogenase/reductase family 29C member 1; pVI-ARLHomo sapiens (human)
Peroxisomal 2,4-dienoyl-CoA reductase [(3E)-enoyl-CoA-producing]pDCR; EC 1.3.1.124; 2,4-dienoyl-CoA reductase 2; Short chain dehydrogenase/reductase family 17C member 1Homo sapiens (human)
Acyl-coenzyme A oxidase-like proteinAcyl-CoA oxidase-like protein; EC 1.3.3.-Homo sapiens (human)
2-hydroxyacyl-CoA lyase 1EC 4.1.2.n2; 2-hydroxyphytanoyl-CoA lyase; 2-HPCL; Phytanoyl-CoA 2-hydroxylase 2Homo sapiens (human)

Compounds (34)

CompoundDescription
hydronium ion
Thiamine PyrophosphateThe coenzyme form of Vitamin B1 present in many animal tissues. It is a required intermediate in the PYRUVATE DEHYDROGENASE COMPLEX and the KETOGLUTARATE DEHYDROGENASE COMPLEX.
Succinate
acetic acidProduct of the oxidation of ethanol and of the destructive distillation of wood. It is used locally, occasionally internally, as a counterirritant and also as a reagent. (Stedman, 26th ed)
NADH
ironA metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN.
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
Hydrogen PeroxideA strong oxidizing agent used in aqueous solution as a ripening agent, bleach, and topical anti-infective. It is relatively unstable and solutions deteriorate over time unless stabilized by the addition of acetanilide or similar organic materials.
MagnesiumA metallic element that has the atomic symbol Mg, atomic number 12, and atomic weight 24.31. It is important for the activity of many enzymes, especially those involved in OXIDATIVE PHOSPHORYLATION.
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)
OxygenAn element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration.
propionylcarnitineRN given refers to cpd without isomeric designation
3-oxopristanoyl-coenzyme A
phytanoyl-coenzyme A
hydroxyphytanoyl-coenzyme Aan intermediate in the alpha-oxidation of phytanic acid in rat liver peroxisomes; its formation is enhanced by dioxygenase cofactors
pristanalstructure given in first source
adenosine monophosphateAdenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position.
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)
alpha-Ketoglutarate
formate
carnitineA constituent of STRIATED MUSCLE and LIVER. It is an amino acid derivative and an essential cofactor for fatty acid metabolism.
formyl-coenzyme a
octanoyl-coenzyme A
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.
2-hydroxyhexadecanoic acidRN given refers to cpd without isomeric designation
pristanic acid
Coenzyme A
lignoceroyl-coenzyme A
phytanic acidA 20-carbon branched chain fatty acid. In phytanic acid storage disease (REFSUM DISEASE) this lipid may comprise as much as 30% of the total fatty acids of the plasma. This is due to a phytanic acid alpha-hydroxylase deficiency.
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.
Malonyl Coenzyme AA coenzyme A derivative which plays a key role in the fatty acid synthesis in the cytoplasmic and microsomal systems.
Flavin-Adenine DinucleotideA condensation product of riboflavin and adenosine diphosphate. The coenzyme of various aerobic dehydrogenases, e.g., D-amino acid oxidase and L-amino acid oxidase. (Lehninger, Principles of Biochemistry, 1982, p972)
creolinfrom refined coal tar oils
propionyl-coenzyme aRN given refers to parent cpd