FITC标记的溶质载体家族25成员19抗体
产品名称： FITC标记的溶质载体家族25成员19抗体
英文名称： Anti-SLC25A19/FITC
产品编号： HZ-21222R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-SLC25A19/FITC Conjugated antibody
FITC标记的溶质载体家族25成员19抗体
| 英文名称 | Anti-SLC25A19/FITC |
| 中文名称 | FITC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: 60386 Human 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 |
该基因编码线粒体蛋白，其是溶质载体家族的成员。虽然最初认为这种蛋白是线粒体脱氧核苷酸载体，参与脱氧核苷酸进入线粒体基质的摄取，但进一步的研究表明，这种蛋白代替了线粒体硫胺素焦磷酸盐载体，H运输硫胺素焦磷酸盐进入线粒体。该基因的突变导致小头畸形，阿米什型，一种代谢性疾病，导致严重的先天性小头畸形，严重的2-酮戊二酸中毒，并在第一年内死亡。已经为该基因鉴定了多个编码相同蛋白的选择性剪接变异体。[ RefSeq，JUL 2008 ]