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PHYH Antibody

Datasheet
Code CSB-PA798729
Size US$166
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Image
  • Immunohistochemistry (IHC) - PHYH Antibody
    The image on the left is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using CSB-PA798729(PHYH Antibody) at dilution 1/20, on the right is treated with fusion protein. (Original magnification: ×200)
  • Immunohistochemistry (IHC) - PHYH Antibody
    The image on the left is immunohistochemistry of paraffin-embedded Human breast cancer tissue using CSB-PA798729(PHYH Antibody) at dilution 1/20, on the right is treated with fusion protein. (Original magnification: ×200)
  • Western Blotting(WB) - PHYH Antibody
    Gel: 10%SDS-PAGE, Lysate: 40 μg, Lane 1-2: A549 cells, mouse liver tissue, Primary antibody: CSB-PA798729(PHYH Antibody) at dilution 1/300, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 1 minute
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Product Details

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
Fusion protein of Human PHYH
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated
Isotype
IgG
Purification Method
Antigen affinity purification
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
Form
Liquid
Tested Applications
ELISA,WB,IHC
Recommended Dilution
Application Recommended Dilution
ELISA 1:2000-1:5000
WB 1:500-1:2000
IHC 1:25-1:100
Protocols
ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) 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.
Usage
For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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PHYH Antibodies

PHYH Proteins

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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
  1. 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
  2. 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
  3. Ten novel PHYH mutations found in Refsum disease patients. PMID: 14974078
  4. 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
  5. 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
  6. 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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