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Browse our Phytanoyl-CoA 2-Hydroxylase Proteins (PHYH)

Full name:: Phytanoyl-CoA 2-Hydroxylase Proteins (PHYH)

On www.antibodies-online.com are 7 Phytanoyl-CoA 2-Hydroxylase (PHYH) Proteins from 5 different suppliers available. Additionally we are shipping Phytanoyl-CoA 2-Hydroxylase Antibodies (157) and many more products for this protein. A total of 171 Phytanoyl-CoA 2-Hydroxylase products are currently listed.

Synonyms:: AI256161, AI265699, LN1, Lnap1, PAHX, PHYH1, RD, zgc:110203

list all proteins	Gene Name	GeneID	UniProt
	PHYH	5264	O14832
	PHYH	114209	P57093
	PHYH	16922	O35386
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Relevant Pathways for Phytanoyl-CoA 2-Hydroxylase Proteins

Monocarboxylic Acid Catabolic Process

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More Proteins for Phytanoyl-CoA 2-Hydroxylase Interaction Partners

Human Phytanoyl-CoA 2-Hydroxylase (PHYH) interaction partners

3 heterozygous variants: c.85C>T (p.Pro29Ser), c.135-2A>G, and c.768del63bp (p.Phe257Glnfs*16) were found in a family with Refsum's disease.
substrate specificity of PAHX is broader than expected, so Refsum disease might be characterized by an accumulation of not only phytanic acid but also other 3-alkyl-branched fatty acids
Ten novel PHYH mutations found in Refsum disease patients.
demonstrate that both unprocessed and processed forms are able to hydroxylate a range of CoA derivatives; site-directed mutagenesis was used to support proposals for the identity of the iron binding istes of PAHX
manner in which phytanoyl-CoA 2-hydroxylase (PAHX) binds to iron(II) and 2-oxoglutarate at its active site distinguishes it from that of the other human 2-oxoglutarate (2OG)-dependent oxygenase
In the absence of elevated phytanic acid concentrations, clinical neurologic abnormalities in heterozygous relatives of Refsum patients are not attributable to heterozygosity for PAHX mutations.

Phytanoyl-CoA 2-Hydroxylase (PHYH) Protein Profile

Protein Summary

This gene is a member of the PhyH family and encodes a peroxisomal protein that is involved in the alpha-oxidation of 3-methyl branched fatty acids. Specifically, this protein converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA. Mutations in this gene have been associated with Refsum disease (RD) and deficient protein activity has been associated with Zellweger syndrome and rhizomelic chondrodysplasia punctata. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.

Alternative names and synonyms associated with Phytanoyl-CoA 2-Hydroxylase (PHYH)

phytanoyl-CoA 2-hydroxylase (PHYH)
phytanoyl-CoA hydroxylase-like (LOC478001)
phytanoyl-CoA 2-hydroxylase (phyh)
phytanoyl-CoA 2-hydroxylase (Phyh)
phytanoyl-CoA hydroxylase (Phyh)

AI256161 protein
AI265699 protein
LN1 protein
Lnap1 protein
PAHX protein
PHYH1 protein
RD protein
zgc:110203 protein

Protein level used designations for PHYH

phytanoyl-CoA hydroxylase (Refsum disease) , phytanoyl-CoA 2-hydroxylase , phytanoyl-CoA dioxygenase, peroxisomal , phytanoyl-CoA hydroxylase , phytanic acid oxidase , phytanoil-CoA alpha hydroxylase , phytanoyl-CoA 2 oxoglutarate dioxygenase , phytanoyl-CoA alpha-hydroxylase , lupus nephritis-associated peptide 1

GENE ID	SPECIES
426601	Gallus gallus
450310	Pan troglodytes
478001	Canis lupus familiaris
550521	Danio rerio
695915	Macaca mulatta
100134962	Sus scrofa
100379998	Xenopus (Silurana) tropicalis
100462930	Papio anubis
5264	Homo sapiens
114209	Rattus norvegicus
281400	Bos taurus
16922	Mus musculus

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