PHYH抗体

PHYH抗体产品信息免疫原 : Recombinant Human PHYH protein (Catalog#13368-HNAE)

Antibody Type : Rabbit Polyclonal Antibody ( Antibody Purification Platform )
抗体宿主 : Rabbit IgG
缓冲液 : 0.2 μm filtered solution in PBS, 5% trehalose may be added in some batches. Please read the hardcopy of COA or contact our customer service to confirm the formulation.
制备方法 : Produced in rabbits immunized with purified, recombinant Human PHYH (rh PHYH; Catalog#13368-HNAE; O14832; Ser31-Leu338). Total IgG was purified by Protein A affinity chromatography.
PHYH抗体 Background
PHYH is a member of 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. It is expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle. PHYH has three substrates which are known as phytanoyl-CoA, 2-oxoglutarate, and O2, while its three products are 2-hydroxyphytanoyl-CoA, succinate, and CO2. PHYH 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.
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