PHYH Antibody

Datasheet

Code	CSB-PA017946DSR1HU
Size	US$166
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Image	Western blot All lanes: Phytanoyl-CoA dioxygenase, peroxisomal antibody at 3μg/ml + Mouse liver tissue Secondary Goat polyclonal to rabbit IgG at 1/10000 dilution Predicted band size: 39, 28 kDa Observed band size: 39 kDa
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Target Background

Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) PHYH Polyclonal antibody

Uniprot No.

O14832

Target Names

PHYH

Alternative Names

LN1 antibody; LNAP1 antibody; LNAP1, mouse, homolog of antibody; OTTHUMP00000019131 antibody; OTTHUMP00000019132 antibody; OTTHUMP00000179083 antibody; OTTHUMP00000216226 antibody; PAHX antibody; PAHX_HUMAN antibody; peroxisomal antibody; PhyH antibody; PHYH1 antibody; Phytanic acid oxidase antibody; phytanoil-CoA alpha hydroxylase antibody; phytanoyl CoA 2 hydroxylase antibody; Phytanoyl CoA 2 oxoglutarate dioxygenase antibody; Phytanoyl CoA alpha hydroxylase antibody; Phytanoyl CoA dioxygenase antibody; Phytanoyl CoA dioxygenase peroxisomal antibody; Phytanoyl-CoA alpha-hydroxylase antibody; Phytanoyl-CoA dioxygenase antibody; RD antibody

Raised in

Rabbit

Species Reactivity

Human, Mouse

Immunogen

Recombinant Human Phytanoyl-CoA dioxygenase, peroxisomal protein (1-338AA)

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

Clonality

Polyclonal

Isotype

IgG

Purification Method

Antigen Affinity Purified

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Form

Liquid

Tested Applications

ELISA, WB

Recommended Dilution

Application	Recommended Dilution
WB	1:500-1:5000

Protocols

ELISA Protocol
Western Blotting(WB) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Customer Reviews and Q&A

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Target Background

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). Does not hydroxylate long and very long straight chain acyl-CoAs or 2-methyl- and 4-methyl-branched acyl-CoAs.

Gene References into Functions

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. PMID: 28681609
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 PMID: 12923223
Ten novel PHYH mutations found in Refsum disease patients. PMID: 14974078
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 PMID: 15930519
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 PMID: 16186124
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. PMID: 18612766

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Involvement in disease

Refsum disease (RD)

Subcellular Location

Peroxisome.

Protein Families

PhyH family

Tissue Specificity

Expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle.

Database Links

HGNC: 8940

OMIM: 266500

KEGG: hsa:5264

STRING: 9606.ENSP00000263038

UniGene: Hs.498732

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Antibody FAQs What Is an Antibody? How to Choose an Antibody for Scientific Research? The difference between primary antibody and secondary antibod The Meaning of Irregular Antibody Screening Tag Antibodies, Your Good Partner in Protein Research How to Choose the Loading Control Antibodies?

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