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

G. A. Jansen; S. J. Mihalik; P. A. Watkins; C. Jakobs; H. W. Moser; R. J. Wanders

doi:https://doi.org/10.1016/S0009-8981(97)00259-3

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

G. A. Jansen, S. J. Mihalik, P. A. Watkins, C. Jakobs, H. W. Moser, R. J. Wanders

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Research output: Contribution to journal › Article › Academic › peer-review

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Abstract

Phytanoyl-Coenzyme A hydroxylase is a newly recognized peroxisomal enzyme which catalyses the first step in the alpha-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

Original language	English
Pages (from-to)	203-211
Journal	Clinica chimica acta; international journal of clinical chemistry
Volume	271
Issue number	2
DOIs	https://doi.org/10.1016/S0009-8981(97)00259-3
Publication status	Published - 1998

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https://doi.org/10.1016/S0009-8981(97)00259-3

Cite this

@article{d401f77f039445c2a7814dff6d37745a,

title = "Characterization of phytanoyl-Coenzyme A hydroxylase in human liver and activity measurements in patients with peroxisomal disorders",

abstract = "Phytanoyl-Coenzyme A hydroxylase is a newly recognized peroxisomal enzyme which catalyses the first step in the alpha-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",

author = "Jansen, {G. A.} and Mihalik, {S. J.} and Watkins, {P. A.} and C. Jakobs and Moser, {H. W.} and Wanders, {R. J.}",

year = "1998",

doi = "https://doi.org/10.1016/S0009-8981(97)00259-3",

language = "English",

volume = "271",

pages = "203--211",

journal = "Clinica chimica acta; international journal of clinical chemistry",

issn = "0009-8981",

publisher = "Elsevier",

number = "2",

}

Characterization of phytanoyl-Coenzyme A hydroxylase in human liver and activity measurements in patients with peroxisomal disorders. / Jansen, G. A.; Mihalik, S. J.; Watkins, P. A. et al.
In: Clinica chimica acta; international journal of clinical chemistry, Vol. 271, No. 2, 1998, p. 203-211.

Research output: Contribution to journal › Article › Academic › peer-review

TY - JOUR

T1 - Characterization of phytanoyl-Coenzyme A hydroxylase in human liver and activity measurements in patients with peroxisomal disorders

AU - Jansen, G. A.

AU - Mihalik, S. J.

AU - Watkins, P. A.

AU - Jakobs, C.

AU - Moser, H. W.

AU - Wanders, R. J.

PY - 1998

Y1 - 1998

N2 - Phytanoyl-Coenzyme A hydroxylase is a newly recognized peroxisomal enzyme which catalyses the first step in the alpha-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

AB - Phytanoyl-Coenzyme A hydroxylase is a newly recognized peroxisomal enzyme which catalyses the first step in the alpha-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

U2 - https://doi.org/10.1016/S0009-8981(97)00259-3

DO - https://doi.org/10.1016/S0009-8981(97)00259-3

M3 - Article

C2 - 9565335

SN - 0009-8981

VL - 271

SP - 203

EP - 211

JO - Clinica chimica acta; international journal of clinical chemistry

JF - Clinica chimica acta; international journal of clinical chemistry

IS - 2

ER -