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Anti-PHYH Antibody (3D991) is a Rabbit antibody targeting PHYH. Anti-PHYH Antibody (3D991) can be used in ELISA.
Pack Size | Price | Availability | Quantity |
---|---|---|---|
100 μL | $269 | 7-10 days |
Description | Anti-PHYH Antibody (3D991) is a Rabbit antibody targeting PHYH. Anti-PHYH Antibody (3D991) can be used in ELISA. |
Alias | RD, phytanoyl-CoA 2-hydroxylase, PHYH1, PAHX, LNAP1, LN1 |
Ig Type | Monoclonal Rabbit IgG |
Clone | 3D991 |
Reactivity | Human |
Application | ELISA |
Recommended Dose | ELISA: 1:5000-1:10000 |
Antibody Type | Monoclonal |
Host Species | Rabbit |
Construction | This antibody was obtained from a rabbit immunized with purified, recombinant Human PHYH (rh PHYH; TMPY-03109; O14832; Ser31-Leu338). |
Purification | Protein A |
Appearance | Liquid |
Formulation | 0.2 μm filtered solution in PBS |
Research Background | PHYH belongs to the family of iron(II)-dependent oxygenases, which typically incorporate one atom of dioxygen into the substrate and one atom into the succinate carboxylate group. PHYH is expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle. It converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA. Defects in PHYH can cause Refsum disease (RD). 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. |
Conjucates | Unconjugated |
Immunogen | Recombinant Human PHYH protein (TMPY-03109) |
Antigen Species | Human |
Stability & Storage | Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. Preservative-Free. |
Transport | Shipping with blue ice. |
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