FITC标记的遗传性脉络膜缺乏症相关蛋白抗体
产品名称： FITC标记的遗传性脉络膜缺乏症相关蛋白抗体
英文名称： Anti-CHM/FITC
产品编号： HZ-13911R-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-CHM/FITC Conjugated antibody
FITC标记的遗传性脉络膜缺乏症相关蛋白抗体
| 英文名称 | Anti-CHM/FITC |
| 中文名称 | FITC标记的遗传性脉络膜缺乏症相关蛋白抗体 |
| 别 名 | CHM; Chm; Choroideraemia protein; Choroideremia; DXS540; FLJ38564; GGTA; HSD 32; MGC102710; Rab escort protein 1; Rab geranylgeranyltransferase component A; Rab proteins geranylgeranyltransferase component A 1; RAE1_HUMAN; REP 1; REP-1; REP1; TCD; TCD protein. |
| 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 神经生物学 信号转导 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, |
| 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 | 73kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human CHM |
| 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 component A of the RAB geranylgeranyl transferase holoenzyme. In the dimeric holoenzyme, this subunit binds unprenylated Rab GTPases and then presents them to the catalytic Rab GGTase subunit for the geranylgeranyl transfer reaction. Rab GTPases need to be geranylgeranyled on either one or two cysteine residues in their C-terminus to localize to the correct intracellular membrane. Mutations in this gene are a cause of choroideremia; also known as tapetochoroidal dystrophy (TCD). This X-linked disease is characterized by progressive dystrophy of the choroid, retinal pigment epithelium and retina. Alternative splicing results in multiple transcript variants encoding different isoforms.[provided by RefSeq, Feb 2009] Function: Binds unprenylated Rab proteins, presents it to the catalytic Rab GGTase dimer, and remains bound to it after the geranylgeranyl transfer reaction. The component A is thought to be regenerated by transferring its prenylated Rab back to the donor membrane. Also a pre-formed complex consisting of CHM and the Rab GGTase dimer (RGGT or component B) can bind to and prenylate Rab proteins; this alternative pathway is proposed to be the predominant pathway for Rab protein geranylgeranylation. Subcellular Location: Belongs to the Rab GDI family. DISEASE: Defects in CHM are the cause of choroideremia (CHM) [MIM:303100]. An X-linked recessive disease characterized by a slowly progressive degeneration of the choroid, photoreceptors, and retinal pigment epithelium. Affected males develop night blindness in their teenage years followed by loss of peripheral vision and complete blindness at middle age. Carrier females are generally asymptomatic but funduscopic examination often shows patchy areas of chorioretinal atrophy. Database links: Entrez Gene: 1121 Human Entrez Gene: 12662 Mouse Omim: 300390 Human SwissProt: P24386 Human SwissProt: Q9QXG2 Mouse Unigene: 496449 Human Unigene: 257316 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rAB香叶醛基转移酶全酶的成分A。在二聚全酶中，该亚基结合未肾化的Rab GTPase并将它们呈现给催化Rab GGTase亚基以进行香叶基香叶基转移反应。Rab GTPase需要在其C末端的一个或两个半胱氨酸残基上进行geranylgeranyled，以定位到正确的细胞内膜。这种基因突变是脉络膜白血病的一个原因；也称为脉络膜营养不良（TCD）。这种X-连锁疾病的特征是脉络膜、视网膜色素上皮和视网膜的进行性营养不良。选择性剪接导致编码不同亚型的多个转录变体。〔RefSeq，FEB 2009〕