SLC19A3 Antibody, Biotin conjugated (PACO59380)
- SKU:
- PACO59380
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- Biotin
Frequently bought together:
Description
抗体名: | SLC19A3 Antibody, Biotin conjugated (PACO59380) |
抗体コード: | PACO59380 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human Thiamine transporter 2 protein (191-282AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Biotin |
バックグラウンド: | Mediates high affinity thiamine uptake, probably via a proton anti-port mechanism. Has no folate transport activity. |
シノニム: | Thiamine transporter 2 (ThTr-2) (ThTr2) (Solute carrier family 19 member 3), SLC19A3 |
UniProt Protein Function: | Mediates high affinity thiamine uptake, probably via a proton anti-port mechanism. Has no folate transport activity. |
NCBI Summary: | 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 cognitive disability, 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.[provided by RefSeq, Jan 2010] |
UniProt Code: | Q9BZV2 |
NCBI GenInfo Identifier: | 13376856 |
NCBI Gene ID: | 80704 |
NCBI Accession: | NP_079519.1 |
UniProt Related Accession: | Q9BZV2 |
Molecular Weight: | 55,665 Da |
NCBI Full Name: | thiamine transporter 2 |
NCBI Synonym Full Names: | solute carrier family 19 member 3 |
NCBI Official Symbol: | SLC19A3 |
NCBI Official Synonym Symbols: | BBGD; THMD2; THTR2 |
NCBI Protein Information: | thiamine transporter 2 |
UniProt Protein Name: | Thiamine transporter 2 |
UniProt Synonym Protein Names: | Solute carrier family 19 member 3 |
Protein Family: | Thiamine transporter |
UniProt Gene Name: | SLC19A3 |