Literature summary for 7.6.2.4 extracted from

Violante, S.; Achetib, N.; van Roermund, C.W.T.; Hagen, J.; Dodatko, T.; Vaz, F.M.; Waterham, H.R.; Chen, H.; Baes, M.; Yu, C.; Argmann, C.A.; Houten, S.M.
Peroxisomes can oxidize medium- and long-chain fatty acids through a pathway involving ABCD3 and HSD17B4 (2019), FASEB J., 33, 4355-4364 .

Search for more literature for 7.6.2.4

Cloned(Commentary)

Cloned (Comment)	Organism
ABCD3, recombinant expression in enzyme-deficient CPT2/ABCD3 KO cells	Homo sapiens

Protein Variants

Protein Variants	Comment	Organism
additional information	construction of ABCD3 single-KO and CPT2/ABCD3 double-KO cell lines, which have undetectable ABCD3 protein levels	Homo sapiens

Localization

Localization	Comment	Organism	GeneOntology No.	Textmining
peroxisomal membrane	-	Homo sapiens	5778	-

Metals/Ions

Metals/Ions	Comment	Organism	Structure
Mg2+	required	Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates	Organism	Comment (Nat. Sub.)	Natural Products	Comment (Nat. Pro.)	Rev.	Reac.
ATP + H2O + C12-carnitine[side 1]	Homo sapiens	-	ADP + phosphate + C12-carnitine[side 2]	-	?
ATP + H2O + C16-carnitine[side 1]	Homo sapiens	-	ADP + phosphate + C16-carnitine[side 2]	-	?
ATP + H2O + fatty acyl CoA[side 1]	Homo sapiens	-	ADP + phosphate + fatty acyl CoA[side 2]	-	?
ATP + H2O + lauroyl-CoA[side 1]	Homo sapiens	-	ADP + phosphate + lauroyl-CoA[side 2]	-	?
ATP + H2O + palmitoyl-CoA[side 1]	Homo sapiens	-	ADP + phosphate + palmitoyl-CoA[side 2]	-	?
additional information	Homo sapiens	peroxisomes accept the CoA and carnitine ester of C12:0 andC16:0 as substrate in a mechanism possibly involving ABCD3. Production of CO2 and acid-soluble products from[1-14C]C16-carnitine. Concentrations of the C16:1-, C16-, C18:2-, C18:1-, and C18-carnitines and the ratio (C16+C18:1) in CPT2 inhibited cells, overview	?	-	?

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	P28288	-	-

Source Tissue

Source Tissue	Comment	Organism	Textmining
HEK-293 cell	-	Homo sapiens	-

Substrates and Products (Substrate)

Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
ATP + H2O + C10-carnitine[side 1]	low activity	Homo sapiens	ADP + phosphate + C10-carnitine[side 2]	-	?
ATP + H2O + C12-carnitine[side 1]	-	Homo sapiens	ADP + phosphate + C12-carnitine[side 2]	-	?
ATP + H2O + C16-carnitine[side 1]	-	Homo sapiens	ADP + phosphate + C16-carnitine[side 2]	-	?
ATP + H2O + fatty acyl CoA[side 1]	-	Homo sapiens	ADP + phosphate + fatty acyl CoA[side 2]	-	?
ATP + H2O + lauroyl-CoA[side 1]	-	Homo sapiens	ADP + phosphate + lauroyl-CoA[side 2]	-	?
ATP + H2O + palmitoyl-CoA[side 1]	-	Homo sapiens	ADP + phosphate + palmitoyl-CoA[side 2]	-	?
additional information	peroxisomes accept the CoA and carnitine ester of C12:0 andC16:0 as substrate in a mechanism possibly involving ABCD3. Production of CO2 and acid-soluble products from[1-14C]C16-carnitine. Concentrations of the C16:1-, C16-, C18:2-, C18:1-, and C18-carnitines and the ratio (C16+C18:1) in CPT2 inhibited cells, overview	Homo sapiens	?	-	?

Synonyms

Synonyms	Comment	Organism
ABCD3	-	Homo sapiens
ATP-binding cassette sub-family D member 3	UniProt	Homo sapiens

Cofactor

Cofactor	Comment	Organism	Structure
ATP	-	Homo sapiens

General Information

General Information	Comment	Organism
metabolism	the peroxisomal import of fatty acids is mediated by 3 ATP-binding cassette transporters (ABCD1, -2, and -3). Transport mediated by ABCD3 is crucial in the peroxisomal degradation of medium-chain fatty acids and might be involved in the production of CO2 and acid-soluble products from C16-carnitine. Mitochondrial FAO inhibition with etomoxir does not lead to significant accumulation of any acylcarnitine species	Homo sapiens
physiological function	the D-bifunctional protein (HSD17B4) and the peroxisomal ABC transporter ABCD3 are essential in peroxisomal oxidation of lauric and palmitic acid, besides mitochondrial carnitine palmitoyltransferase (CPT)2 (EC 2.3.1.21), leading to the production of peroxisomal acylcarnitine intermediates. Peroxisomes accept acyl-CoAs and oxidize acylcarnitines in a similar biochemical pathway as the mitochondria. Peroxisomal fatty acid beta-oxidation (FAO) is important when mitochondrial FAO is defective or overloaded. The peroxisomal import of fatty acids is mediated by 3 ATP-binding cassette transporters (ABCD1, -2, and -3). Transport mediated by ABCD3 is crucial in the peroxisomal degradation of medium-chain fatty acids. The peroxisomal ABC transporters transport acyl-CoA intermediates and C12/C16-carnitines, peroxisomes accept the CoA and carnitine ester of C12:0 and C16:0 as substrate in a mechanism possibly involving ABCD3	Homo sapiens

Use of this online version of BRENDA is free under the CC BY 4.0 license. See terms of use for full details.

Release 2023.1 (January 2023)