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

Characterization of phytanoyl-Coenzyme A hydroxylase in human liver and activity measurements in patients with peroxisomal disorders.
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人肝脏中植物酰辅酶 A 羟化酶的表征以及过氧化物酶体疾病患者的活性测量。

DOI:
10.1016/s0009-8981(97)00259-3
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发表时间:
1998
期刊:
Clinica chimica acta; international journal of clinical chemistry
影响因子:
--
通讯作者:
Wanders,RJ
Wanders,RJ
中科院分区:
--
文献类型:
--
作者:
Jansen,GA;Mihalik,SJ;Watkins,PA;Jakobs,C;Moser,HW;Wanders,RJ

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Phytanoyl-Coenzyme A hydroxylase is a newly recognized peroxisomal enzyme which catalyses the first step in the α-oxidation of phytanoyl-Coenzyme A. Since measurement of this enzyme activity in human liver homogenate is of great importance especially in relation to inherited diseases in which this enzyme activity is deficient, we have studied its characteristics in human liver. The results described in this paper show that optimal activity measurements require preformed phytanoyl-Coenzyme A plus 2-oxoglutarate, Fe2+and ascorbate. The conditions developed can be used to determine phytanoyl-Coenzyme A hydroxylase activity in human liver homogenates which is of utmost importance not only for the diagnosis of patients, but also for the purification of the enzyme from various sources.
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DOI: --
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