Human PHYH Recombinant Protein (RPES0130)
- SKU:
- RPES0130
- Product type:
- Recombinant Protein
- Host species:
- E.coli
- Reactivity:
- Human
Frequently bought together:
Description
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Human PHYH Recombinant Protein
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.
| Product Name: | Human PHYH Recombinant Protein (RPES0130) |
| Product Code: | RPES0130 |
| Size: | 20µg |
| Species: | Human |
| Expressed Host: | E.coli |
| Synonyms: | LN1,LNAP1,PAHX,PHYH1,RD |
| Accession: | O14832 |
| Sequence: | Ser31-Leu338 |
| Fusion tag: | |
| Endotoxin: | Please contact us for more information. |
| Protein Construction: | A DNA sequence encoding the human PHYH (O14832) (Ser31-Leu338) was expressed, with a N-terminal Met. |
| Purity: | > 80 % as determined by reducing SDS-PAGE. |
| Mol Mass: | 35.6 kDa |
| AP Mol Mass: | 26-32 kDa |
| Formulation: | Lyophilized from sterile 20mM mops, 10% glycerol, 2mM DDT, 1mM EDTA, 0.2mM PMSF, 0.2M NaCl, pH 7.2 |
| Shipping: | This product is provided as lyophilized powder which is shipped with ice packs. |
| Stability and Storage: | Lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8°C for 2-7 days. Aliquots of reconstituted samples are stable at < -20°C for 3 months. |
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