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Rabbit Anti-FLVCR/BF594 Conjugated antibody (bs-12344R-BF594)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-12344R-BF594
英文名稱1 Rabbit Anti-FLVCR/BF594 Conjugated antibody
中文名稱 BF594標記的白血病病毒C亞類受體蛋白FLVCR抗體
別    名 Feline leukemia virus subgroup C cellular receptor; Feline leukemia virus subgroup C receptor; Feline leukemia virus subgroup C receptor related protein 1; Feline leukemia virus subgroup C receptor-related protein 1; FLVC1_HUMAN; FLVCR 1; FLVCR protein; FLVCR1; hFLVCR.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 腫瘤  細胞生物  發(fā)育生物學  干細胞  細菌及病毒  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng)
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 60kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from Human FLVCR
亞    型 IgG
純化方法 affinity purified by Protein A
儲 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:
FLVCR is responsible for the exportation of cytoplasmic heme groups. It is believed that it may protect developing erythroid cells from heme toxicity. Expression of FLVCR in cells will cause susceptibility to FeLV-C (Feline leukemia virus subgroup C) in vitro. FLVCR is found in all hematopoietic tissues, including peripheral blood lymphocytes and fetal liver, and some expression is found in pancreas and kidney. It is down-regulated in haemopoietic progenitor cells undergoing differentiation and hemoglobinization.

Function:
Heme transporter that exports cytoplasmic heme. It can also export coproporphyrin and protoporphyrin IX, which are both intermediate products in the heme biosynthetic pathway. Does not export bilirubin. Heme export depends on the presence of HPX and may be required to protect developing erythroid cells from heme toxicity. Heme export also provides protection from heme or ferrous iron toxicities in liver and brain. Causes susceptibility to FeLV-C in vitro.

Subunit:
Interacts with HPX.

Subcellular Location:
Cell membrane.

Tissue Specificity:
Found all hematopoietic tissues including peripheral blood lymphocytes. Some expression is found in pancreas and kidney.

DISEASE:
Defects in FLVCR1 are the cause of posterior column ataxia with retinitis pigmentosa (PCARP) [MIM:609033]. A neurodegenerative syndrome beginning in infancy with areflexia and retinitis pigmentosa. Nyctalopia (night blindness) and peripheral visual field loss are usually evident during late childhood or teenage years, with subsequent progressive constriction of the visual fields and loss of central retinal function over time. A sensory ataxia caused by degeneration of the posterior columns of the spinal cord results in a loss of proprioceptive sensation that is clinically evident in the second decade of life and gradually progresses. Scoliosis, camptodactyly, achalasia, gastrointestinal dysmotility, and a sensory peripheral neuropathy are variable features of the disease. Affected individuals have no clinical or radiological evidence of cerebral or cerebellar involvement. Note=Defective neuronal heme transmembrane export due to FLVCR1 mutations may abrogate the neuroprotective effects of neuroglobin and initiate an apoptotic cascade that results in the selective degeneration of photoreceptors in the neurosensory retina and sensory neurons in the posterior spinal cord.

Similarity:
Belongs to the major facilitator superfamily.
Feline leukemia virus subgroup C receptor (TC 2.A.1.28.1) family.

Database links:

Entrez Gene: 28982 Human

Omim: 609144 Human

SwissProt: Q9Y5Y0 Human

Unigene: 592816 Human

Unigene: 7055 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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