Product Name	FANCC
Chinese Name	范可尼综合征相关蛋白FANCC抗体
Alias	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.
Research Area	Cell biology  Epigenetics
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Human,  (predicted: Mouse, Rat, Dog, Horse, )
Applications	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 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	63kDa
Cellular localization	The nucleus cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human FANCC: 61-160/558
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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
Product Picture	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 (SL13140R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.

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