溶质载体家族25成员19抗体
规格：1mg/1ml
英文名： SLC25A19
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
分子量： 36kDa
储存液：Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human SLC25A19
交叉反应：Human, Mouse,
细胞定位：
溶质载体家族25成员19抗体产品介绍：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. 溶质载体家族25成员19抗体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: 60386 Human溶质载体家族25成员19抗体 Omim: 606521 Human SwissProt: Q9HC21 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
溶质载体家族25成员19抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 信号转导 转运蛋白
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid