| 產(chǎn)品編號 | bs-8702R |
| 英文名稱(chēng) | SLC19A3 Rabbit pAb |
| 中文名稱(chēng) | 溶質(zhì)載體家族19成員3抗體 |
| 別 名 | Solute carrier family 19 member 3; Thiamine transporter 2; thTr 2; THTR2. |
| 研究領(lǐng)域 | 細胞生物 神經(jīng)生物學(xué) 信號轉導 轉運蛋白 跨膜蛋白 |
| 抗體來(lái)源 | Rabbit |
| 克隆類(lèi)型 | Polyclonal |
| 克 隆 號 | |
| 交叉反應 | Mouse (predicted: Human,Rat) |
| 產(chǎn)品應用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 56 kDa |
| 檢測分子量 | |
| 細胞定位 | 細胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human SLC19A3: 261-360/496 <Extracellular> |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | Preservative: 0.02% Proclin300, Constituents: 1% BSA, 0.01M PBS, pH7.4. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
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.[provided by RefSeq, Jan 2010] Function: Mediates high affinity thiamine uptake, propably via a proton anti-port mechanism. Has no folate transport activity. Subcellular Location: Plasma membrane. Tissue Specificity: Widely expressed but most abundant in placenta, kidney and liver. DISEASE: The disease is caused by mutations affecting the gene represented in this entry. Disease description:An autosomal recessive metabolic disorder characterized by episodic encephalopathy, often triggered by febrile illness, presenting as confusion, seizures, external ophthalmoplegia, dysphagia, and sometimes coma and death. If untreated, encephalopathies can result in permanent dystonia. Brain imaging may show characteristic bilateral lesions of the basal ganglia. Similarity: Belongs to the reduced folate carrier (RFC) transporter (TC 2.A.48) family. SWISS: Q9BZV2 Gene ID: 80704 Database links: Entrez Gene: 80704 Human Omim: 606152 Human SwissProt: Q9BZV2 Human Unigene: 221597 Human |
| 產(chǎn)品圖片 |
Sample:
Liver (Mouse) Lysate at 40 ug
Primary: Anti-SLC19A3 (bs-8702R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 56 kD
Observed band size: 56 kD
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