Species: | Rabbit |
Applications: | WB IHC IF |
Immunogen Range: | KLH conjugated synthetic peptide derived from human FANCC |
Clonality: | Polyclonal Antibody |
Isotype: | IgG |
GENE ID: | 2176 |
Swiss Prot: | |
Synonyms: | 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. |
Purification: | Purified by Protein A. |
Storage: | Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at -20℃ for 12 months |
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). |
Caculated MW: | / |
Observed MW: | Refer to Figures |
Applications: |
WB 1:100-1:1000 IHC 1:100-1:500 IF 1:50-1:200 |
Reacitivity: | Human, Mouse, Rat |