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货号： bs-8595R 基本售价： 780.0 元 规格： 50ul
- 规格：50ul
- 价格：780.00元
- 规格：100ul
- 价格：1380.00元
- 规格：200ul
- 价格：2200.00元
产品信息
- 产品编号
- bs-8595R
- 英文名称
- C9orf72
- 中文名称
- 9号染色体开放阅读框72抗体
- 别 名
- chromosome 9 open reading frame 72; CI072_HUMAN; MGC23980; Uncharacterized protein C9orf72.
- 规格价格
- 50ul/780元购买 100ul/1380元购买 200ul/2200元购买 大包装/询价
- 说 明 书
- 50ul 100ul 200ul
- 研究领域
- 细胞生物 染色质和核信号 神经生物学 转录调节因子
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse,
- 产品应用
- WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 Flow-Cyt=1ug/test ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 53kDa
- 细胞定位
- 细胞核 细胞浆
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human C9orf72:391-481/481
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- 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.
- PubMed
- PubMed
- 产品介绍
- background:
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.
- 产品图片
Sample:
293T(Human) Cell Lysate at 40 ug
Primary: Anti-C9orf72 (bs-8595R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 53 kD
Observed band size: 53 kD
Sample:
Kidney(Rat) Lysate at 40 ug
Primary: Anti-C9orf72 (bs-8595R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 53 kD
Observed band size: 53 kD
Tissue/cell: human liver carcinoma; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-Arginase 1 Polyclonal Antibody, Unconjugated(bs-8585R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
U-937 cells were fixed with 4% PFA for 10min at room temperature,permeabilized with 90% ice-cold methanol for 20 min at room temperature,and incubated in 5% BSA blocking buffer for 30 min at room temperature. Cells were then stained with C9orf72 Antibody(bs-8595R) at 1:100 dilution in blocking buffer and incubated for 30 min at room temperature, washed twice with 2%BSA in PBS, followed by secondary antibody incubation for 40 min at room temperature. Acquisitions of 20,000 events were performed.Cells stained with primary antibody (green), and isotype control (orange).