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9號染色體開放閱讀框72抗體
  • 產(chǎn)品貨號：
    BN41966R
  • 中文名稱：
    9號染色體開放閱讀框72抗體
  • 英文名稱：
    Rabbit anti-C9orf72 Polyclonal antibody
  • 品牌：
    Biorigin

  • 貨號

    產(chǎn)品規(guī)格

    售價

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  • BN41966R-50ul

    50ul

    ￥1486.00

    交叉反應(yīng)：Human,Mouse,Rat(predicted:Chicken,Dog,Pig,Cow,Horse) 推薦應(yīng)用：WB,IHC-P,IHC-F,ICC,IF,Flow-Cyt,ELISA

  • BN41966R-100ul

    100ul

    ￥2360.00

    交叉反應(yīng)：Human,Mouse,Rat(predicted:Chicken,Dog,Pig,Cow,Horse) 推薦應(yīng)用：WB,IHC-P,IHC-F,ICC,IF,Flow-Cyt,ELISA

  • BN41966R-200ul

    200ul

    ￥3490.00

    交叉反應(yīng)：Human,Mouse,Rat(predicted:Chicken,Dog,Pig,Cow,Horse) 推薦應(yīng)用：WB,IHC-P,IHC-F,ICC,IF,Flow-Cyt,ELISA

產(chǎn)品描述

英文名稱C9orf72
中文名稱9號染色體開放閱讀框72抗體
別    名chromosome 9 open reading frame 72; CI072_HUMAN; MGC23980; Uncharacterized protein C9orf72.  
研究領(lǐng)域細胞生物  染色質(zhì)和核信號  神經(jīng)生物學(xué)  轉(zhuǎn)錄調(diào)節(jié)因子  
抗體來源Rabbit
克隆類型Polyclonal
交叉反應(yīng)Human, Mouse, Rat,  (predicted: Chicken, Dog, Pig, Cow, Horse, )
產(chǎn)品應(yīng)用WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1ug/test ICC=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復(fù)）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量53kDa
細胞定位細胞核 細胞漿 
性    狀Liquid
濃    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human C9orf72:391-481/481 
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Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
產(chǎn)品介紹Chromosome 9 consists of about 145 million bases and 4% of the human genome and encodes nearly 900 genes. Considered to play a role in gender determination, deletion of the distal portion of 9p can lead to development of male to female sex reversal, the phenotype of a female with a male X,Y genotype. Hereditary hemorrhagic telangiectasia, which is characterized by harmful vascular defects, is associated with the chromosome 9 gene encoding endoglin protein, ENG. Familial dysautonomia is also associated with chromosome 9 though through the gene IKBKAP. Notably, chromosome 9 encompasses the largest interferon family gene cluster. Chromosome 9 is partnered with chromosome 22 in the translocation leading to the aberrant production of BCR-ABL fusion protein often found in leukemias. The C9orf72 gene product has been provisionally designated C9orf72 pending further characterization. There are two isoforms of C9orf72 that are produced as a result of alternative splicing events.

Subcellular Location:
Cytoplasm. Nucleus. Note=Detected in the cytoplasm of neurons from post mortem brain tissue (PubMed:21944778). Detected in the nucleus in fibroblasts (PubMed:21944779).

Tissue Specificity:
Both isoforms are widely expressed, including kidney, lung, liver, heart, testis and several brain regions, such as cerebellum. Also expressed in the frontal cortex and in lymphoblasts (at protein level).

DISEASE:
Defects in C9orf72 are the cause of frontotemporal dementia and/or amyotrophic lateral sclerosis (FTDALS) [MIM:105550]. An autosomal dominant neurodegenerative disorder characterized by adult onset of frontotemporal dementia and/or amyotrophic lateral sclerosis in an affected individual. There is high intrafamilial variation. Frontotemporal dementia is characterized by frontal and temporal lobe atrophy associated with neuronal loss, gliosis, and dementia. Patients exhibit progressive changes in social, behavioral, and/or language function. Amyotrophic lateral sclerosis is characterized by the death of motor neurons in the brain, brainstem, and spinal cord, resulting in fatal paralysis. Note=Caused by a large expansion of a GGGGCC hexanucleotide within the first C9orf72 intron located between the first and the second non-coding exons. The expansion leads to the loss of transcription of one of the two transcripts encoding isoform 1 and to the formation of nuclear RNA foci.

SWISS:
Q96LT7

Gene ID:
203228

Database links:

Entrez Gene: 203228 Human

Entrez Gene: 73205 Mouse

Entrez Gene: 313155 Rat

Omim: 614260 Human

SwissProt: Q96LT7 Human

SwissProt: Q6DFW0 Mouse

SwissProt: Q66HC3 Rat

Unigene: 493639 Human

Unigene: 331544 Mouse

Unigene: 233897 Rat



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