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Mouse Anti-PHYH Recombinant Antibody (S210-5) (CBMAB-R0181-CN)

This product is a Mouse antibody that recognizes PHYH. The antibody S210-5 can be used for immunoassay techniques such as: ICC, WB.
See all PHYH antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
S210-5
Antibody Isotype
IgG1
Application
ICC, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Buffer
PBS, pH 7.4, 50% Glycerol
Preservative
0.1% Sodium azide
Concentration
1 mg/mL
Epitope
aa. 1-338

Target

Full Name
phytanoyl-CoA 2-hydroxylase
Introduction
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. [provided by RefSeq, Jul 2008]
Entrez Gene ID
5264
UniProt ID
O14832
Alternative Names
Phytanoyl-CoA 2-Hydroxylase; Phytanoyl-CoA Alpha-Hydroxylase; Phytanic Acid Oxidase; EC 1.14.11.18; PAHX; Phytanoyl-CoA Hydroxylase (Refsum Disease); Phytanoyl-CoA 2 Oxoglutarate Dioxygenase; Phytanoyl-CoA Dioxygenase, Peroxisomal; Phytanoil-CoA Alpha Hydroxylase;
Function
Catalyzes the 2-hydroxylation of not only racemic phytanoyl-CoA and the isomers of 3-methylhexadecanoyl-CoA, but also a variety of other mono-branched 3-methylacyl-CoA esters (with a chain length of at least seven carbon atoms) and straight-chain acyl-CoA esters (with a chain length longer than four carbon atoms) (PubMed:9326939, PubMed:10744784, PubMed:12031666, PubMed:12923223).
Does not hydroxylate long and very long straight chain acyl-CoAs or 2-methyl- and 4-methyl-branched acyl-CoAs (PubMed:10744784, PubMed:12923223).
Biological Process
2-oxobutyrate catabolic processIEA:Ensembl
2-oxoglutarate metabolic processManual Assertion Based On ExperimentIDA:CAFA
Fatty acid alpha-oxidationManual Assertion Based On ExperimentIDA:UniProtKB
Isoprenoid metabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Methyl-branched fatty acid metabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Cellular Location
Peroxisome
Involvement in disease
Refsum disease (RD):
A rare autosomal recessive peroxisomal disorder characterized by the accumulation of the branched-chain fatty acid, phytanic acid, in blood and tissues. Cardinal clinical features are retinitis pigmentosa, peripheral neuropathy, cerebellar ataxia, and elevated protein levels in the cerebrospinal fluid (CSF). Half of all patients exhibit generalized, mild to moderate ichthyosis resembling ichthyosis vulgaris. Less constant features are nerve deafness, anosmia, skeletal abnormalities, cataracts and cardiac impairment.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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