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[alpha-Oxidation of 3-methyl-branched fatty acids: unraveling of a pathway].
Verh K Acad Geneeskd Belg. 2006; 68(3):199-221; discussion 221-2.VK

Abstract

Peroxisomes have an important role in lipid metabolism e.g. beta-oxidation of long and very long chain fatty acids, 2-methyl-branched fatty acids, dicarboxylic fatty acids, prostanoids and bile acid intermediates, and synthesis of ether lipids. Also the process of alpha-oxidation of 3-methyl-branched fatty acids, with phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) as the best known example, occurs in peroxisomes. alpha-Oxidation is a process in which fatty acids are shortened by one carbon atom. The alpha-oxidation sequence of 3-methyl-branched fatty acids starts with an activation to the corresponding CoA-ester. Subsequently this acyl-CoA-ester undergoes a 2-hydroxylation by the peroxisomal phytanoyl-CoA hydroxylase (PAHX). In a third step the peroxisomal 2-hydroxyphytanoyl-CoA lyase (2-HPCL) splits the carbon carbon bond of the 2-hydroxy-intermediate into a 2-methyl(n-1)aldehyde and formyl-CoA, which is subsequently converted to formate and CO2. Finally the aldehyde is dehydrogenated by an aldehyde dehydrogenase to the corresponding acid, which, after its conversion to the acyl-CoA ester, can be a substrate for beta-oxidation. 2-HPCL is the first thiamine pyrophosphate dependent peroxisomal enzyme in mammals. Apart from 2-hydroxy-3-methylacyl-CoAs also 2-hydroxyacyl-CoAs are substrates for this enzyme. This indicates that the 2-hydroxy function but not the 3-methyl function of acyl-CoA esters is needed for 2-HPCL-activity. Long and very long chain 2-hydroxy fatty acids are constituents of brain cerebrosides and sulfatides, which mainly occur in myelin.

Authors+Show Affiliations

Afdeling Farmacologie, Departement Moleculaire Celbiologie, Faculteit Geneeskunde, KULeuven, Herestraat 49, B 3000 Leuven.

Pub Type(s)

Congress

Language

dut

PubMed ID

16796018

Citation

Casteels, M. "[alpha-Oxidation of 3-methyl-branched Fatty Acids: Unraveling of a Pathway]." Verhandelingen - Koninklijke Academie Voor Geneeskunde Van Belgie, vol. 68, no. 3, 2006, pp. 199-221; discussion 221-2.
Casteels M. [alpha-Oxidation of 3-methyl-branched fatty acids: unraveling of a pathway]. Verh K Acad Geneeskd Belg. 2006;68(3):199-221; discussion 221-2.
Casteels, M. (2006). [alpha-Oxidation of 3-methyl-branched fatty acids: unraveling of a pathway]. Verhandelingen - Koninklijke Academie Voor Geneeskunde Van Belgie, 68(3), 199-221; discussion 221-2.
Casteels M. [alpha-Oxidation of 3-methyl-branched Fatty Acids: Unraveling of a Pathway]. Verh K Acad Geneeskd Belg. 2006;68(3):199-221; discussion 221-2. PubMed PMID: 16796018.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [alpha-Oxidation of 3-methyl-branched fatty acids: unraveling of a pathway]. A1 - Casteels,M, PY - 2006/6/27/pubmed PY - 2006/8/1/medline PY - 2006/6/27/entrez SP - 199-221; discussion 221-2 JF - Verhandelingen - Koninklijke Academie voor Geneeskunde van Belgie JO - Verh K Acad Geneeskd Belg VL - 68 IS - 3 N2 - Peroxisomes have an important role in lipid metabolism e.g. beta-oxidation of long and very long chain fatty acids, 2-methyl-branched fatty acids, dicarboxylic fatty acids, prostanoids and bile acid intermediates, and synthesis of ether lipids. Also the process of alpha-oxidation of 3-methyl-branched fatty acids, with phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) as the best known example, occurs in peroxisomes. alpha-Oxidation is a process in which fatty acids are shortened by one carbon atom. The alpha-oxidation sequence of 3-methyl-branched fatty acids starts with an activation to the corresponding CoA-ester. Subsequently this acyl-CoA-ester undergoes a 2-hydroxylation by the peroxisomal phytanoyl-CoA hydroxylase (PAHX). In a third step the peroxisomal 2-hydroxyphytanoyl-CoA lyase (2-HPCL) splits the carbon carbon bond of the 2-hydroxy-intermediate into a 2-methyl(n-1)aldehyde and formyl-CoA, which is subsequently converted to formate and CO2. Finally the aldehyde is dehydrogenated by an aldehyde dehydrogenase to the corresponding acid, which, after its conversion to the acyl-CoA ester, can be a substrate for beta-oxidation. 2-HPCL is the first thiamine pyrophosphate dependent peroxisomal enzyme in mammals. Apart from 2-hydroxy-3-methylacyl-CoAs also 2-hydroxyacyl-CoAs are substrates for this enzyme. This indicates that the 2-hydroxy function but not the 3-methyl function of acyl-CoA esters is needed for 2-HPCL-activity. Long and very long chain 2-hydroxy fatty acids are constituents of brain cerebrosides and sulfatides, which mainly occur in myelin. SN - 0302-6469 UR - https://www.unboundmedicine.com/medline/citation/16796018/[alpha_Oxidation_of_3_methyl_branched_fatty_acids:_unraveling_of_a_pathway]_ DB - PRIME DP - Unbound Medicine ER -
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