Studies on the specificity of unprocessed and mature forms of phytanoyl-CoA 2-hydroxylase and mutation of the iron binding ligands

Studies on the specificity of unprocessed and mature forms of phytanoyl-CoA 2-hydroxylase and mutation of the iron binding ligands

The mature form of phytanoyl-coenzyme A 2-hydroxylase (PAHX), a nonheme Fe(II)- and 2-oxoglutarate-dependent oxygenase, catalyzes the α-hydroxylation of phytanoyl-CoA within peroxisomes. Mutations in PAHX result in some forms of adult Refsum's disease. Unprocessed PAHX (pro-PAHX) contains an N-...

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Bibliographic Details
Main Authors: Timothy Searls, Danica Butler, Winnie Chien, Mridul Mukherji, Matthew D. Lloyd, Christopher J. Schofield
Format: Article
Language:English
Published: Elsevier 2005-08-01
Series:Journal of Lipid Research
Subjects:
chemical rescue
oxygenase
2-oxoglutarate
phytanoyl-coenzyme A 2-hydroxylase
phytanic acid
Refsum's disease
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520329655

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