Title : Identification of PAHX, a Refsum disease gene.

Pub. Date : 1997 Oct

PMID : 9326939






1 Functional Relationships(s)
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Compound Name
Protein Name
Organism
1 Human PAHX is targetted to peroxisomes, requires the PTS2 receptor for peroxisomal localization, interacts with the PTS2 receptor in the yeast two-hybrid assay and has intrinsic phytanoyl-CoA alpha-hydroxylase activity that requires the dioxygenase cofactor iron and cosubstrate 2-oxoglutarate. Iron phytanoyl-CoA 2-hydroxylase Homo sapiens