产品说明
摘要
C9orf72 antibody detects the C9orf72 protein, which is 481 amino acids in length and has a predicted molecular weight of 54 kDa. The accumulation of abnormal nucleotide repeats in the C9orf72 gene is the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), accounting for approximately 34.2% and 25.9% of cases according to van Blitterswijk et al. (PMID: 23160421).
产品应用
应用说明
*最佳稀释倍数与浓度应由研究人员确认
实验应用 |
稀释倍数 |
1:100-1:1000 |
Assay dependent |
1:100-1:1000 |
WB: 免疫印迹
ICC/IF: 细胞染色
IHC-P: 石蜡切片
IHC-Fr: 冰冻切片
预期分子量
阳性对照
C9orf72-transfected 293T , Neuro2A , NIH-3T3 , Raw264.7 , C2C12 , mouse brain , U87-MG , SK-N-SH , IMR32 , SK-N-AS , 293T
预测种属反应
Bovine, Rhesus Monkey(>80% identity)
产品属性
形式
Liquid
存储溶液
PBS
存放说明
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
浓度
1 mg/ml (Please refer to the vial label for the specific concentration.)
免疫原种属
Human
免疫原
Recombinant protein encompassing a sequence within the N-terminus region of human C9orf72. The exact sequence is proprietary.
纯化方式
Affinity purified by Protein A.
偶联
Unconjugated
RRID
AB_2784545
注意事项
仅供实验室使用。不适用于人类或动物的任何临床,治疗或诊断用途。不适合动物或人类食用。
相关讯息
别名
C9orf72-SMCR8 complex subunit , ALSFTD , DENNL72 , FTDALS , FTDALS1
细胞定位
Nucleus,Cytoplasm,Endosome,Lysosome,Secreted,Cell projection, dendrite
功能与背景
The protein encoded by this gene plays an important role in the regulation of endosomal trafficking, and has been shown to interact with Rab proteins that are involved in autophagy and endocytic transport. Expansion of a GGGGCC repeat from 2-22 copies to 700-1600 copies in the intronic sequence between alternate 5' exons in transcripts from this gene is associated with 9p-linked ALS (amyotrophic lateral sclerosis) and FTD (frontotemporal dementia) (PMID: 21944778, 21944779). Studies suggest that hexanucleotide expansions could result in the selective stabilization of repeat-containing pre-mRNA, and the accumulation of insoluble dipeptide repeat protein aggregates that could be pathogenic in FTD-ALS patients (PMID: 23393093). Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2016]
数据库
研究领域
產品图片
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GTX634482 WB Image
Non-transfected (–) and transfected (+) 293T whole cell extracts (60 μg) were separated by 10% SDS-PAGE, and the membrane was blotted with C9orf72 antibody [GT1553] (GTX634482) diluted at 1:500. The HRP-conjugated anti-mouse IgG antibody (GTX213111-01) was used to detect the primary antibody, and the signal was developed with Trident ECL plus-Enhanced.
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GTX634482 IHC-P Image
C9orf72 antibody [GT1553] detects C9orf72 protein at cytoplasm by immunohistochemical analysis.Sample: Paraffin-embedded mouse hippocampus.C9orf72 stained by C9orf72 antibody [GT1553] (GTX634482) diluted at 1:100.Antigen Retrieval: Citrate buffer, pH 6.0, 15 min
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GTX634482 WB Image
Mouse tissue extract (50 μg) was separated by 10% SDS-PAGE, and the membrane was blotted with C9orf72 antibody [GT1553] (GTX634482) diluted at 1:1000. The HRP-conjugated anti-mouse IgG antibody (GTX213111-01) was used to detect the primary antibody.
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GTX634482 IHC-P Image
C9orf72 antibody [GT1553] detects C9orf72 protein at cytoplasm by immunohistochemical analysis.Sample: Paraffin-embedded mouse hippocampus.C9orf72 stained by C9orf72 antibody [GT1553] (GTX634482) diluted at 1:100.Antigen Retrieval: Citrate buffer, pH 6.0, 15 min
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GTX634482 IHC-P Image
C9orf72 antibody [GT1553] detects C9orf72 protein at cytoplasm by immunohistochemical analysis.Sample: Paraffin-embedded mouse hippocampus.C9orf72 stained by C9orf72 antibody [GT1553] (GTX634482) diluted at 1:100.Antigen Retrieval: Citrate buffer, pH 6.0, 15 min
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GTX634482 ICC/IF Image
C9orf72 antibody [GT1553] detects C9orf72 protein at cytoplasmic granule by immunofluorescent analysis.Sample: DIV9 rat E18 primary hippocampal neuron cells were fixed in 4% paraformaldehyde at RT for 15 min.Green: C9orf72 stained by C9orf72 antibody [GT1553] (GTX634482) diluted at 1:250.Red: Tau, an Axon marker, stained by Tau antibody (GTX130462) diluted at 1:500.Blue: Fluoroshield with DAPI (GTX30920).
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GTX634482 WB Image
Non-transfected (–) and transfected (+) 293T whole cell extracts (30 μg) were separated by 10% SDS-PAGE, and the membrane was blotted with C9orf72 antibody [GT1553] (GTX634482) diluted at 1:5000. The HRP-conjugated anti-mouse IgG antibody (GTX213111-01) was used to detect the primary antibody.
|
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GTX634482 WB Image
Various whole cell extracts (30 μg) were separated by 10% SDS-PAGE, and the membrane was blotted with C9orf72 antibody [GT1553] (GTX634482) diluted at 1:500. The HRP-conjugated anti-mouse IgG antibody (GTX213111-01) was used to detect the primary antibody.
|
|
GTX634482 WB Image
Various whole cell extracts (30 μg) were separated by 10% SDS-PAGE, and the membrane was blotted with C9orf72 antibody [GT1553] (GTX634482) diluted at 1:1000. The HRP-conjugated anti-mouse IgG antibody (GTX213111-01) was used to detect the primary antibody.
|
|
GTX634482 WB Image
Various whole cell extracts (30 μg) were separated by 10% SDS-PAGE, and the membrane was blotted with C9orf72 antibody [GT1553] (GTX634482) diluted at 1:500. The HRP-conjugated anti-mouse IgG antibody (GTX213111-01) was used to detect the primary antibody.
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GTX634482 WB Image
The data was published in the journal Acta Neuropathol Commun in 2019. PMID: 31651360
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GTX634482 WB Image
The data was published in the journal Acta Neuropathol Commun in 2019. PMID: 31651360
|
|
GTX634482 WB Image
The data was published in the journal Acta Neuropathol Commun in 2019. PMID: 31651360
|
|
GTX634482 IHC-P Image
The data was published in the journal Acta Neuropathol Commun in 2019. PMID: 31651360
|
|
GTX634482 WB Image
The data was published in the journal Acta Neuropathol Commun in 2019. PMID: 31651360
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文献引用
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