FITC标记的MUTED蛋白抗体
产品名称： FITC标记的MUTED蛋白抗体
英文名称： Anti-MUTED/FITC
产品编号： HZ-19114R-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-MUTED/FITC Conjugated antibody
FITC标记的MUTED蛋白抗体
| 英文名称 | Anti-MUTED/FITC |
| 中文名称 | FITC标记的MUTED蛋白抗体 |
| 别 名 | Biogenesis of lysosomal organelles complex 1, subunit 5, muted; Biogenesis of lysosome related organelles complex 1 subunit 5; BLOC 1 subunit 5; BLOS5; MU; MUTED; Muted homolog; Muted protein homolog; MUTED_HUMAN; Protein Muted homolog. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 细胞生物 信号转导 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Dog, Cow, Horse, |
| 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 | 21kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human MUTED |
| 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: The BLOC-1 complex is required for normal biogenesis of lysosome-related organelles, such as platelet dense granules and melanosomes. Plays a role in intracellular vesicle trafficking. Subunit: Interacts with BLOC1S4, DTNBP1/BLOC1S7 and PI4K2A (By similarity). Component of the biogenesis of lysosome-related organelles complex 1 (BLOC-1) composed of BLOC1S1, BLOC1S2, BLOC1S3, BLOC1S4, BLOC1S5, BLOC1S6, DTNBP1/BLOC1S7 and SNAPIN/BLOC1S8. Octamer composed of one copy each BLOC1S1, BLOC1S2, BLOC1S3, BLOC1S4, BLOC1S5, BLOC1S6, DTNBP1/BLOC1S7 and SNAPIN/BLOC1S8. The BLOC-1 complex associates with the AP-3 protein complex and membrane protein cargos. Interacts with BLOC1S6. Similarity: Belongs to the Muted family. Database links: Entrez Gene: 63915 Human Entrez Gene: 17828 Mouse Omim: 607289 Human SwissProt: Q8TDH9 Human SwissProt: Q8R015 Mouse Unigene: 719272 Human Unigene: 261554 Mouse 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。多个交替剪接变异体，编码相同的蛋白质，已被鉴定为该基因。[ RefSeq，JUL 2008 ]