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Products:Signal Transduction >> Protein Trafficking >> Organelle Proteins
Overview
Product name
Anti-PHYH antibody
See all PHYH products (3) ...
Description
Mouse monoclonal to PHYH
Tested applications
Cross reactivity
Reacts with
Human
Immunogen
Recombinant full length protein, corresponding to amino acids 1-339 of Human PHYH
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage buffer
Preservative: None
PBS, pH 7.2
Concentration
Concentration information loading...
Purity
Protein G purified
Clonality
Monoclonal
Isotype
IgG1
Light chain type
kappa
Research areas
Cancer >> Cancer Metabolism >> Metabolic signaling pathway >> Metabolism of lipids and lipoproteins
Signal Transduction >> Metabolism >> Lipid metabolism
Signal Transduction >> Metabolism >> Vitamins / Minerals
Neuroscience >> Neurology process >> Neurogenesis
Neuroscience >> Sensory System >> Auditory system
Neuroscience >> Sensory System >> Visual system
Signal Transduction >> Protein Trafficking >> Organelle Proteins
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Western blot - PHYH antibody (ab55585)
(enlarge)
Applications
Show applications key
Our Abpromise guarantee covers the use of ab55585 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
- 1 Image
WB
Application notes
WB: Use at a concentration of 1-5 µg/ml.
This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
Target
Function
Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
Tissue specificity
Expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle.
Pathway
Lipid metabolism; fatty acid metabolism.
Involvement in disease
Defects in PHYH are a cause of Refsum disease (RD) [MIM:266500]. RD is an autosomal recessive disorder characterized clinically by a tetrad of abnormalities: retinitis pigmentosa, peripheral neuropathy, cerebellar ataxia, and elevated protein levels in the cerebrospinal fluid (CSF). Patients exhibit accumulation of the branched-chain fatty acid, phytanic acid, in blood and tissues. Less constant features are nerve deafness, anosmia, skeletal abnormalities, ichthyosis, cataracts and cardiac impairment. Manifestations of the disease appear in the second or third decade of life.
Sequence similarities
Belongs to the PhyH family.
Cellular localization
Peroxisome.
Target information above from: UniProt accessionO14832
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).
Alternative names
- peroxisomal antibody
- LN1 antibody
- LNAP1 antibody
see all
Database links
- Entrez Gene: 5264 Human
- Omim: 602026 Human
- SwissProt: O14832 Human
- Unigene: 498732 Human
Anti-PHYH antibody images:
Western blot - PHYH antibody (ab55585)
Western blot against tagged recombinant protein immunogen using ab55585 PHYH antibody at 1ug/ml. Predicted band size of immunogen is 63 kDa
References for Anti-PHYH antibody (ab55585)
ab55585 has not yet been referenced specifically in any publications.
Publishing research using ab55585? Please let us know so that we can cite the reference in this datasheet
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"