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Rabbit Anti-FANCA/APC Conjugated antibody (bs-13138R-APC)
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢(xún)價(jià)
產(chǎn)品編號(hào) bs-13138R-APC
英文名稱(chēng) Rabbit Anti-FANCA/APC Conjugated antibody
中文名稱(chēng) APC標(biāo)記的范可尼貧血組蛋白A抗體
別    名 FA 1; FA; FA H; FA1; FAA; FACA; FAH; Fanca; FANCA_HUMAN; FANCH; Fanconi anemia complementation group A; Fanconi anemia complementation group H; Fanconi anemia group A protein; Fanconi anemia type 1; MGC75158; Protein FACA.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢(xún)價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 細(xì)胞生物  發(fā)育生物學(xué)  細(xì)胞周期蛋白  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Chicken, Rabbit, )
產(chǎn)品應(yīng)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 160kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FANCA
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 background:
Fanconi anemia (FA) is an autosomal recessive disorder characterized by bone marrow failure, birth defects and chromosomal instability. At the cellular level, FA is characterized by spontaneous chromosomal breakage and a unique hypersensitivity to DNA cross-linking agents. At least eight complementation groups (A-G) have been identified and six FA genes (for subtypes A, C, D2, E, F and G) have been cloned. The FA proteins lack sequence homologies or motifs that could point to a molecular function. The cellular accumulation of FA proteins, including FANCA and FANCG, is subject to regulation by TNF alpha signaling. Phosphorylation of FANC (Fanconi anemia complementation group) proteins is thought to be important for the function of the FA pathway. FANCA, also known as FACA and FANCH, associates with the Brm-related gene 1 (BRG1) product, a subunit of the SWI/SNF complex which remodels chromatin structure through a DNA-dependent ATPase activity. FANCA is mainly expressed in lymphoid tissues, testis and ovary. The amino-terminal region of the FANCA protein is required for FANCG binding, FANCC binding, nuclear localization and functional activity of the complex. The human FANCA gene maps to chromosome 16q24.3 and encodes a 1,455 amino acid protein.

Function:
DNA repair protein that may operate in a postreplication repair or a cell cycle checkpoint function. May be involved in interstrand DNA cross-link repair and in the maintenance of normal chromosome stability

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. In complex with FANCF, FANCG and FANCL, but not with FANCC, nor FANCE, interacts with HES1; this interaction may be essential for the stability and nuclear localization of FA core complex proteins. The complex with FANCC and FANCG may also include EIF2AK2 and HSP70. Interacts with FAAP20/C1orf86; interaction is direct.

Subcellular Location:
Nucleus. Cytoplasm. Note=The major form is nuclear. The minor form is cytoplasmic.

Post-translational modifications:
Phosphorylated upon DNA damage, probably by ATM or ATR. Phosphorylation is required for the formation of the nuclear complex. Not phosphorylated in cells derived from groups A, B, C, E, F, G, and H.

DISEASE:
Defects in FANCA 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.

Similarity:
Nucleus. Cytoplasm. The major form is nuclear. The minor form is cytoplasmic.

Database links:

Entrez Gene: 2175 Human

Entrez Gene: 14087 Mouse

Omim: 607139 Human

SwissProt: O15360 Human

SwissProt: Q9JL70 Mouse

Unigene: 290154 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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