FITC标记的范可尼综合征相关蛋白FANCC抗体
产品名称： FITC标记的范可尼综合征相关蛋白FANCC抗体
英文名称： Anti-FANCC/FITC
产品编号： HZ-13140R-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-FANCC/FITC Conjugated antibody
FITC标记的范可尼综合征相关蛋白FANCC抗体
| 英文名称 | Anti-FANCC/FITC |
| 中文名称 | FITC标记的范可尼综合征相关蛋白FANCC抗体 |
| 别 名 | bA80I15.1; FA 3; FA3; FAC; FACC; FANCC; FANCC_HUMAN; Fanconi anemia complementation group C; Fanconi anemia complementation group C protein; Fanconi anemia group C protein; Fanconi pancytopenia type 3; FLJ14675; Protein FACC. |
| 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 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, 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 | 63kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human FANCC |
| 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: Fanconi anemia (FA) is an autosomal recessive disorder characterized by bone marrow failure, birth defects and chromsomal instability (1,2). The FA Group C complementation group gene encodes the protein FANCC, which is located in both cytoplasmic and nuclear compartments. FANCC is expressed in a cell cycle-dependent manner, with the lowest levels at the G1/S boundary and the highest levels in the M-phase. The FANCC protein interacts with other FA complementation group proteins as well as non-FA proteins (3). A human a spectrin II (designated aSpIIs) acts as a scaffold to enhance interactions between FANCC and FANCA to form a nuclear complex (4,5). Another binding partner of FANCC is the BTB/POZ domain containing protein FAZF, which is a transcriptional repressor (6). In hematopoietic cells expressing mutant FANCC, PKR is constitutively phosphorylated and has increased binding affinity for double-stranded RNA (7,8), which suggests that FANCC indirectly suppresses the activity of PKR. These cells are also apoptotic and are hypersensitive to IFNg and TNFa (8). In addition, FANCC protein is involved in the activation of STAT1 through receptors for at least three hematopoietic growth and survival factors (8). Function: DNA repair protein that may operate in a postreplication repair or a cell cycle checkpoint function. May be implicated in interstrand DNA cross-link repair and in the maintenance of normal chromosome stability. Upon IFNG induction, may facilitate STAT1 activation by recruiting STAT1 to IFNGR1. Subunit: Belongs to the multisubunit FA complex composed of FANCA, FANCB, FANCC, FANCE, FANCF, FANCG, FANCL/PHF9 and FANCM. This complex may also include HSP70. The complex is not found in FA patients. Interacts with ZBTB32. Upon IFNG induction, interacts with STAT1. Interacts with CDK1. Interacts with EIF2AK2; interaction between FA variants and EIF2AK2 may lead to augmented EIF2AK2 activation and cell death. Subcellular Location: Nucleus. Cytoplasm. The major form is nuclear. The minor form is cytoplasmic. Tissue Specificity: Ubiquitous. DISEASE: Defects in FANCC are the cause of Fanconi anemia complementation group C (FANCC) [MIM:227645]. A disorder affecting all bone marrow elements and resulting in anemia, leukopenia and thrombopenia. It is associated with cardiac, renal and limb malformations, dermal pigmentary changes, and a predisposition to the development of malignancies. At the cellular level it is associated with hypersensitivity to DNA-damaging agents, chromosomal instability (increased chromosome breakage) and defective DNA repair. Database links: UniProtKB/Swiss-Prot: Q00597.1 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
范可尼贫血（FA）是一种常染色体隐性遗传病，以骨髓衰竭、出生缺陷和染色体不稳定（1，2）为特征。FA C互补组基因编码FANCC蛋白，该蛋白位于细胞质和核室。FANCC以细胞周期依赖性的方式表达，G1/S边界最低，M期最高。FANC蛋白与其他FA互补组蛋白以及非FA蛋白相互作用（3）。人α光谱蛋白II(命名为aSpIIs)作为支架增强FANCC与FANCA之间的相互作用以形成核复合物(4,5)。FANCC的另一结合伙伴是BTB/POZ结构域，含有蛋白FAZF，它是转录阻遏物（6）。在表达突变型FANCC的造血细胞中，PKR被组成性磷酸化，与双链RNA（7，8）的结合亲和力增加，提示FANCC间接抑制PKR的活性。这些细胞也是凋亡的，对IFNG和TNFA（8）过敏。此外，FANCC蛋白通过至少三种造血生长因子和生存因子的受体参与STAT1的激活(8)。