Product Name:
PHYH/PAHX
Product Number:
ab-nn306-1
Target Full Name: Phytanoyl-CoA dioxygenase, peroxisomal
Target Alias: PAHX; peroxisomal; PhyH; PHYH1; Phytanic acid oxidase; phytanoil-CoA alpha hydroxylase; phytanoyl CoA 2 hydroxylase; Phytanoyl CoA 2 oxoglutarate dioxygenase; Phytanoyl CoA alpha hydroxylase; Phytanoyl CoA dioxygenase; Phytanoyl CoA dioxygenase peroxisomal; Phytanoyl-CoA alpha-hydroxylase; Phytanoyl-CoA dioxygenase; RD; LN1; LNAP1
Product Type Specific: PHYH/PAHX pan-specific antibody
Antibody Code: NN306-1
Antibody Target Type: Pan-specific
Protein UniProt: O14832
Protein SigNET: PHYH/PAHX
Antibody Type: Monoclonal
Antibody Host Species: Mouse
Antibody Ig Isotype Clone: IgG1
Antibody Immunogen Source: Fusion protein with full-length of human PhyH
Antibody Immunogen Description: Corresponds to amino acid residues M1-L338. Rat: 78% identity (264/338 amino acids identical). Mouse: 78% identity (264/338 amino acids identical).
Production Method: Protein G purified
Antibody Modification: Unconjugated. Contact KInexus if you are interest in having the antibody biotinylated or coupled with fluorescent dyes.
Antibody Concentration: 1 mg/ml
Storage Buffer: Phosphate buffered saline pH 7.4, 50% glycerol, 0.1% sodium azide
Storage Conditions: For long term storage, keep frozen at -40°C or lower. Stock solution can be kept at +4°C for more than 3 months. Avoid repeated freeze-thaw cycles.
Product Use: Western blotting | ICC/Immunofluorescence
Antibody Dilution Recommended: WB (1:1000); optimal dilutions for assays should be determined by the user.
Antibody Potency: In mouse brain lysates, this antibody detects a ~40 kDa protein by Western blotting.
Antibody Species Reactivity: This antibody detects the target protein in the following species due to conservation of amino acid sequence: Human | Chimpanzee | Rhesus macaque | Rat | Mouse.
Antibody Positive Control: 1 µg/ml of SMC-472 was sufficient for detection of PHYH/PAHX in 20 µg of rat brain lysate by colorimetric immunoblot analysis using Goat anti-mouse IgG:HRP as the secondary antibody.
Antibody Specificity: Very high
Scientific Background: PHYH (phytanoyl-CoA hydroxylase; PAHX) is a peroxisomal enzyme that catalyzes the crucial first step in the alpha-oxidation of 3-methyl-branched fatty acids, specifically breaking down phytanoyl-CoA into 2-hydroxyphytanoyl-CoA. PAHX is expressed in liver, kidney, and T-cells. It is localized within the peroxisomes, which are cell structures responsible for breaking down fatty acids. It breaks down dietary phytanic acid (found in dairy and meat) which cannot be processed by normal beta-oxidation due to a 3-methyl group. PHYH is found in the liver, kidney, and other tissues, with high expression levels found in adults within these organs, as well as the heart, gut, and brain. Mutations in the PHYH gene lead to deficient enzyme activity, resulting in the accumulation of phytanic acid in tissues and plasma, causing symptoms like retinitis pigmentosa, sensory neuropathy, and ataxia. Deficient PHYH activity has also been linked to Zellweger syndrome and rhizomelic chondrodysplasia punctata. This description may include information annotated by UniProt and/or Google AI.