SLC19A3, GST-Tag, Recombinant (THTR2, SLC19A3, BBGD, THMD2, Thiamine Transporter 2)
Catalog No : USB-500971
482.77€
0.00€
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| Product name | SLC19A3, GST-Tag, Recombinant (THTR2, SLC19A3, BBGD, THMD2, Thiamine Transporter 2) | ||
|---|---|---|---|
| Catalog No | USB-500971 | ||
| Supplier’s Catalog No | 500971 | ||
| Supplier | US Biologicals | ||
| Source antigen | E. coli, Recombinant | ||
| Reactivity | |||
| Cross reactivity | |||
| Applications | |||
| Molecular weight | 43 | ||
| Storage | -20°C | ||
|---|---|---|---|
| Other names | |||
| Grade | Lysate | ||
| Purity | Lysate | ||
| Form | Supplied as a lyophilized powder in Tris-HCl, pH 6.8, 0.2M DTT, 4% SDS, 0.2% bromophenol blue, 20% sucrose. Reconstitute with 200ul sterile dH2O. | ||
| Reactivity life | 6 months | ||
| Note | For reserch purpose only | ||
| Purity | Lysate | ||
| Description | This gene encodes a ubiquitously expressed transmembrane thiamine transporter that lacks folate transport activity. Mutations in this gene cause biotin-responsive basal ganglia disease (BBGD); a recessive disorder manifested in childhood that progresses to chronic encephalopathy, dystonia, quadriparesis, and death if untreated. Patients with BBGD have bilateral necrosis in the head of the caudate nucleus and in the putamen. Administration of high doses of biotin in the early progression of the disorder eliminates pathological symptoms while delayed treatment results in residual paraparesis, mild mental retardation, or dystonia. Administration of thiamine is ineffective in the treatment of this disorder. Experiments have failed to show that this protein can transport biotin. Mutations in this gene also cause a Wernicke's-like encephalopathy. Source: Partial length protein corresponding to of SLC19A3, fused to GST-Tag at N-terminal, expressed in E. coli. Applications: Suitable for use in Western Blot. Other applications not tested. Recommended Dilution: Optimal dilutions to be determined by the researcher. Molecular Weight: ~43kD Storage and Stability: Lyophilized powder may be stored at -20°C. Stable for 12 months after receipt at -20°C. Reconstitute with sterile ddH2O. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Reconstituted product is stable for 6 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer. | ||
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