产品中心
当前位置：首页>产品中心Anti-SLC25A19/AF350
货号： bs-21222R-AF350 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-21222R-AF350
- 英文名称
- Anti-SLC25A19/AF350
- 中文名称
- AF350标记的溶质载体家族25成员19抗体
- 别 名
- DNC; MCPHA; Mitochondrial thiamine pyrophosphate carrier; Mitochondrial uncoupling protein 1; MUP1; Solute carrier family 25 (mitochondrial deoxynucleotide carrier) member 19; Solute carrier family 25 (mitochondrial thiamine pyrophosphate carrier) member 19; Solute carrier family 25 member 19; TPC.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 细胞生物 信号转导 转运蛋白
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 36kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 2mg/1ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human SLC25A19
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
This gene encodes a mitochondrial protein that is a member of the solute carrier family. Although this protein was initially thought to be the mitochondrial deoxynucleotide carrier involved in the uptake of deoxynucleotides into the matrix of the mitochondria, further studies have demonstrated that this protein instead functions as the mitochondrial thiamine pyrophosphate carrier, which transports thiamine pyrophosphates into mitochondria. Mutations in this gene cause microcephaly, Amish type, a metabolic disease that results in severe congenital microcephaly, severe 2-ketoglutaric aciduria, and death within the first year. Multiple alternatively spliced variants, encoding the same protein, have been identified for this gene. [provided by RefSeq, Jul 2008]
Function:
Mitochondrial transporter mediating uptake of thiamine pyrophosphate (ThPP) into mitochondria.
Tissue Specificity:
Expressed in all tissues examined except for placenta. Highest levels in colon, kidney, lung, testis, spleen, and brain.
DISEASE:
The disease is caused by mutations affecting the gene represented in this entry.
Disease description:A disorder characterized by severe congenital microcephaly and severe 2-ketoglutaric aciduria leading to death within the first year.
The disease is caused by mutations affecting the gene represented in this entry.
Disease description:A disease characterized by recurrent episodes of flaccid paralysis and encephalopathy associated with bilateral striatal necrosis and chronic progressive polyneuropathy.
Similarity:
Belongs to the mitochondrial carrier (TC 2.A.29) family.
Contains 3 Solcar repeats.
Database links:Entrez Gene: 60386Human
Omim: 606521Human
SwissProt: Q9HC21Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.