Phytanic acid alpha-oxidation in man: identification of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal enzyme with normal activity in Zellweger syndrome.

作者信息

Jansen G A, Denis S, Verhoeven N M, Jakobs C, Wanders R J

机构信息

University of Amsterdam, Academic Medical Centre, Department of Clinical Chemistry, Emma Children's Hospital, The Netherlands.

出版信息

J Inherit Metab Dis. 2000 Jun;23(4):421-4. doi: 10.1023/a:1005672406773.

DOI:10.1023/a:1005672406773

PMID:10896309

Abstract

摘要

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Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism.

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本文引用的文献

Phytanic acid alpha-oxidation: identification of 2-hydroxyphytanoyl-CoA lyase in rat liver and its localisation in peroxisomes.

Biochim Biophys Acta. 1999 Sep 22;1440(2-3):176-82. doi: 10.1016/s1388-1981(99)00126-2.

The metabolism of phytanic acid and pristanic acid in man: a review.

J Inherit Metab Dis. 1998 Oct;21(7):697-728. doi: 10.1023/a:1005476631419.

Characterization of phytanoyl-Coenzyme A hydroxylase in human liver and activity measurements in patients with peroxisomal disorders.

Clin Chim Acta. 1998 Mar 23;271(2):203-11. doi: 10.1016/s0009-8981(97)00259-3.

Phytanic acid alpha-oxidation: decarboxylation of 2-hydroxyphytanoyl-CoA to pristanic acid in human liver.

J Lipid Res. 1997 Oct;38(10):2062-70.

Refsum disease is caused by mutations in the phytanoyl-CoA hydroxylase gene.

Nat Genet. 1997 Oct;17(2):190-3. doi: 10.1038/ng1097-190.

Formation of a 2-methyl-branched fatty aldehyde during peroxisomal alpha-oxidation.

FEBS Lett. 1997 Aug 4;412(3):643-5. doi: 10.1016/s0014-5793(97)00856-9.

Resolution of the phytanic acid alpha-oxidation pathway: identification of pristanal as product of the decarboxylation of 2-hydroxyphytanoyl-CoA.

Biochem Biophys Res Commun. 1997 Aug 8;237(1):33-6. doi: 10.1006/bbrc.1997.7066.

Phytanoyl-coenzyme A hydroxylase deficiency -- the enzyme defect in Refsum's disease.

N Engl J Med. 1997 Jul 10;337(2):133-4. doi: 10.1056/NEJM199707103370215.

Phytanic acid alpha-oxidation in rat liver peroxisomes. Production of alpha-hydroxyphytanoyl-CoA and formate is enhanced by dioxygenase cofactors.

Eur J Biochem. 1995 Sep 1;232(2):545-51. doi: 10.1111/j.1432-1033.1995.545zz.x.

A major pathway for the mammalian oxidative degradation of phytanic acid.

Biochim Biophys Acta. 1969 Jun 10;176(4):720-39. doi: 10.1016/0005-2760(69)90253-7.

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