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Phytanoyl-CoA 2-Hydroxylase Proteins (PHYH)

PHYH 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. Additionally we are shipping Phytanoyl-CoA 2-Hydroxylase Antibodies (92) and many more products for this protein.

list all proteins Gene Name GeneID UniProt
PHYH 5264 O14832
PHYH 114209 P57093
PHYH 16922 O35386
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Top Phytanoyl-CoA 2-Hydroxylase Proteins at antibodies-online.com

Showing 7 out of 7 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
HOST_Escherichia coli (E. coli) Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Escherichia coli (E. coli) Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Wheat germ Human GST tag 10 μg Log in to see 9 Days
$405.71
Details
HOST_Escherichia coli (E. coli) Human Un-conjugated 100 μg Log in to see 16 Days
$437.80
Details
Yeast Cow His tag   1 mg Log in to see 56 to 66 Days
$2,817.83
Details
Yeast Rat His tag   1 mg Log in to see 56 to 66 Days
$2,821.50
Details
HOST_Human Human Un-conjugated   20 μg Log in to see 9 to 11 Days
$785.40
Details

PHYH Proteins by Origin and Source

Origin Expressed in Conjugate
Human , ,
,
Rat (Rattus)

Mouse (Murine)

More Proteins for Phytanoyl-CoA 2-Hydroxylase (PHYH) Interaction Partners

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

  1. 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

  2. Ten novel PHYH mutations found in Refsum disease patients.

  3. 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

  4. 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

  5. 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.

Gene names and symbols associated with PHYH

  • phytanoyl-CoA 2-hydroxylase (PHYH)
  • 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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