Price range: $268.00 through $328.00
Background:
This gene encodes a member of the Fanconi anemia complementation group B. This protein is assembled into a nucleoprotein complex that is involved in the repair of DNA lesions. Mutations in this gene can cause chromosome instability and VACTERL syndrome with hydrocephalus. [provided by RefSeq, Apr 2016],
| Size | 50μL/100μL |
| Target | FANCB |
| Reactivity | Human, Mouse |
| Applications | WB |
| MW(Calculated) | 94kD |
| MW(Observed) | |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-2000 |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | 1 mg/ml |
| Clonality | Polyclonal |
| Clone Number | |
| Immunogen | Synthesized peptide derived from human FANCB AA range: 585-635 |
| Specificity | This antibody detects endogenous levels of FANCB at Human/Mouse |
| Gene Name | FANCB |
| Protein Name | FANCB |
| Other Name | |
| Species | Human |
| Gene ID | 2187 |
| Uniprot | Q8NB91, |
| Species.1 | Mouse |
| Gene ID.1 | 237211 |
| Uniprot.1 | Q5XJY6 |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | Nucleus . |
| Tissue Expression | |
| Signaling_pathway | |
| Research Areas | >>Fanconi anemia pathway |
| Function | Disease:Defects in FANCB are a cause of Fanconi anemia (FA) [MIM:227650]. FA is a genetically heterogeneous, autosomal recessive disorder characterized by progressive pancytopenia, a diverse assortment of congenital malformations, 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.,Disease:Defects in FANCB are the cause of cause of Fanconi anemia complementation group B (FANCB) [MIM:300514]; also called Fanconi pancytopenia type 2 (FA2).,Disease:Defects in FANCB are the cause of X-linked VACTERL-H (XVACTERL-H) [MIM:314390]; also known as X-linked VACTERL association with hydrocephalus syndrome. VACTERL is an acronym for vertebral anomalies, anal atresia, cardiac malformations, tracheoesophageal fistula, renal anomalies (urethral atresia with hydronephrosis), and limb anomalies (hexadactyly, humeral hypoplasia, radial aplasia, and proximally placed thumb). Some cases of VACTERL-H are associated with increased chromosome breakage and rearrangement.,Function:DNA repair protein required for FANCD2 ubiquitination.,sequence Caution:Contaminating sequence. Potential poly-A sequence.,subunit:Belongs to the multisubunit FA complex composed of FANCA, FANCB, FANCC, FANCE, FANCF, FANCG, FANCL/PHF9 and FANCM. The complex is not found in FA patients., |
