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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

相似文献

1
Phytanic acid alpha-oxidation in man: identification of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal enzyme with normal activity in Zellweger syndrome.
J Inherit Metab Dis. 2000 Jun;23(4):421-4. doi: 10.1023/a:1005672406773.
2
Phytanic acid alpha-oxidation: identification of 2-hydroxyphytanoyl-CoA lyase in rat liver and its localisation in peroxisomes.植烷酸α-氧化：大鼠肝脏中2-羟基植烷酰辅酶A裂解酶的鉴定及其在过氧化物酶体中的定位。
Biochim Biophys Acta. 1999 Sep 22;1440(2-3):176-82. doi: 10.1016/s1388-1981(99)00126-2.
3
Phytanoyl-CoA hydroxylase is present in human liver, located in peroxisomes, and deficient in Zellweger syndrome: direct, unequivocal evidence for the new, revised pathway of phytanic acid alpha-oxidation in humans.植烷酰辅酶A羟化酶存在于人类肝脏中，位于过氧化物酶体，在 Zellweger 综合征中缺乏：这是人类植烷酸α-氧化新修订途径的直接、明确证据。
Biochem Biophys Res Commun. 1996 Dec 4;229(1):205-10. doi: 10.1006/bbrc.1996.1781.
4
Phytanic acid oxidation in man: identification of a new enzyme catalysing the formation of 2-ketophytanic acid from 2-hydroxyphytanic acid and its deficiency in the Zellweger syndrome.人体内植烷酸氧化：一种催化从2-羟基植烷酸形成2-酮植烷酸的新酶的鉴定及其在齐-韦综合征中的缺乏。
J Inherit Metab Dis. 1995;18(2):201-3. doi: 10.1007/BF00711766.
5
Identification of pristanal dehydrogenase activity in peroxisomes: conclusive evidence that the complete phytanic acid alpha-oxidation pathway is localized in peroxisomes.过氧化物酶体中降植烷酸脱氢酶活性的鉴定：植烷酸α-氧化完整途径定位于过氧化物酶体的确凿证据。
Biochem Biophys Res Commun. 2001 May 11;283(3):674-9. doi: 10.1006/bbrc.2001.4835.
6
Phytanic acid alpha-oxidation in rat liver peroxisomes. Production of alpha-hydroxyphytanoyl-CoA and formate is enhanced by dioxygenase cofactors.大鼠肝脏过氧化物酶体中的植烷酸α-氧化。双加氧酶辅因子可增强α-羟基植烷酰辅酶A和甲酸的生成。
Eur J Biochem. 1995 Sep 1;232(2):545-51. doi: 10.1111/j.1432-1033.1995.545zz.x.
7
2-Hydroxyphytanic acid oxidase activity in rat and human liver and its deficiency in the Zellweger syndrome.
Biochim Biophys Acta. 1994 Nov 29;1227(3):177-82. doi: 10.1016/0925-4439(94)90092-2.
8
Identification of pristanoyl-CoA oxidase and phytanic acid decarboxylation in peroxisomes and mitochondria from human liver: implications for Zellweger syndrome.
J Inherit Metab Dis. 1991;14(3):349-52. doi: 10.1007/BF01811700.
9
Phytanic acid alpha-oxidation and complementation analysis of classical Refsum and peroxisomal disorders.植烷酸α-氧化及经典型雷夫叙姆病和过氧化物酶体疾病的互补分析
Hum Genet. 1989 Jan;81(2):175-81. doi: 10.1007/BF00293897.
10
Purification, molecular cloning, and expression of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal thiamine pyrophosphate-dependent enzyme that catalyzes the carbon-carbon bond cleavage during alpha-oxidation of 3-methyl-branched fatty acids.2-羟基植烷酰辅酶A裂解酶的纯化、分子克隆及表达，该酶是一种过氧化物酶体中依赖硫胺焦磷酸的酶，在3-甲基支链脂肪酸的α-氧化过程中催化碳-碳键的裂解。
Proc Natl Acad Sci U S A. 1999 Aug 31;96(18):10039-44. doi: 10.1073/pnas.96.18.10039.

引用本文的文献

1
Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism.遗传性过氧化物酶体脂肪酸代谢紊乱的生化与遗传学
J Lipid Res. 2010 Oct;51(10):2863-95. doi: 10.1194/jlr.R005959. Epub 2010 Jun 17.

本文引用的文献

1
Phytanic acid alpha-oxidation: identification of 2-hydroxyphytanoyl-CoA lyase in rat liver and its localisation in peroxisomes.植烷酸α-氧化：大鼠肝脏中2-羟基植烷酰辅酶A裂解酶的鉴定及其在过氧化物酶体中的定位。
Biochim Biophys Acta. 1999 Sep 22;1440(2-3):176-82. doi: 10.1016/s1388-1981(99)00126-2.
2
The metabolism of phytanic acid and pristanic acid in man: a review.人体中植烷酸和降植烷酸的代谢：综述
J Inherit Metab Dis. 1998 Oct;21(7):697-728. doi: 10.1023/a:1005476631419.
3
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.
4
Phytanic acid alpha-oxidation: decarboxylation of 2-hydroxyphytanoyl-CoA to pristanic acid in human liver.
J Lipid Res. 1997 Oct;38(10):2062-70.
5
Refsum disease is caused by mutations in the phytanoyl-CoA hydroxylase gene.Refsum病由植烷酰辅酶A羟化酶基因突变引起。
Nat Genet. 1997 Oct;17(2):190-3. doi: 10.1038/ng1097-190.
6
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.
7
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.
8
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.
9
Phytanic acid alpha-oxidation in rat liver peroxisomes. Production of alpha-hydroxyphytanoyl-CoA and formate is enhanced by dioxygenase cofactors.大鼠肝脏过氧化物酶体中的植烷酸α-氧化。双加氧酶辅因子可增强α-羟基植烷酰辅酶A和甲酸的生成。
Eur J Biochem. 1995 Sep 1;232(2):545-51. doi: 10.1111/j.1432-1033.1995.545zz.x.
10
A major pathway for the mammalian oxidative degradation of phytanic acid.植烷酸哺乳动物氧化降解的主要途径。
Biochim Biophys Acta. 1969 Jun 10;176(4):720-39. doi: 10.1016/0005-2760(69)90253-7.