| 产品编号 | bs-13140R |
| 英文名称 | FANCC Rabbit pAb |
| 中文名称 | 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. |
| 研究领域 | 细胞生物 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 克 隆 号 | |
| 交叉反应 | Human, (predicted: Mouse, Rat, Dog, Horse, ) |
| 产品应用 | WB=1:500-2000 ELISA=1:5000-10000 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. |
| 理论分子量 | 63 kDa |
| 检测分子量 | |
| 细胞定位 | 细胞核 细胞浆 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human FANCC: 61-160/558 |
| 亚 型 | |
| 纯化方法 | affinity purified by Protein A |
| 缓 冲 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存条件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 产品介绍 |
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. SWISS: Q00597 Gene ID: 2176 Database links: Entrez Gene: 2176 Human Omim: 613899 Human SwissProt: Q00597 Human Unigene: 494529 Human |
| 产品图片 |
Paraformaldehyde-fixed, paraffin embedded (human colon carcinoma); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (FANCC) Polyclonal Antibody, Unconjugated (bs-13140R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
