C9ORF72 抗体 (AA 425-481)
Quick Overview for C9ORF72 抗体 (AA 425-481) (ABIN7540524)
抗原
See all C9ORF72 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 425-481
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Supplier Product No.
- 200-901-mh2
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Supplier
- Rockland
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原理
- C9orf72 Antibody
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交叉反应 (详细)
- This affinity purified antibody is directed against human C9orf72.
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纯化方法
- This product is an IgY fraction antibody purified from monospecific chicken egg yolks by a multi-step process which includes selective precipitation and salt fractionation followed by extensive dialysis against the buffer stated above.
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过滤
- Sterile filtered
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免疫原
- Anti-C9orf72 antibody was prepared from eggs of chickens laid after repeated immunizations with a synthetic peptide corresponding to a C-terminal portion of human C9orf72 surrounding aa 425-481 conjugated to Keyhole Limpet Hemocyanin (KLH).
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亚型
- IgG
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应用备注
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ELISA_Dilution: 1:10,000 - 1:50,000
Immunohistochemistry_Dilution: 1:200
Flow_Cytometry_Dilution: 1:20
IF_Microscopy_Dilution: 5 μg/mL
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说明
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Anti-C9orf72 Antibody has been tested in Immunohistochemistry, Immunofluorescence, and Flow Cytometry. Western blot not recommended. Positive control used: Human cerebellum in IHC, U2-OS in IF, MCF7 in FLOW.
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限制
- 仅限研究用
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状态
- Liquid
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缓冲液
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Buffer: 0.002 M Sodium Phosphate, 0.15 M Sodium Chloride, pH 7.0
Stabilizer: None
Preservative: 0.01 % (w/v) Sodium Azide -
储存液
- Sodium azide
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注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- 4 °C,-20 °C
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储存方法
- Store vial at -20° C prior to opening. Aliquot contents and freeze at -20° C or below for extended storage. Avoid cycles of freezing and thawing. Centrifuge product if not completely clear after standing at room temperature. This product is stable for several weeks at 4° C as an undiluted liquid. Dilute only prior to immediate use.
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有效期
- 12 months
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- C9ORF72 (Chromosome 9 Open Reading Frame 72 (C9ORF72))
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别名
- C9orf72
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背景
- Chicken Anti-Chromosome 9 Open Reading Frame 72 Antibody, Chicken Anti-C9orf72 Antibody, Guanine Nucleotide Exchange, Protein C9orf72, DENNL72, FTDALS1, ALSFTD, FTDALS,C9orf72 (Chromosome 9 Open Reading Frame 72) 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. It is a component of the C9orf72-SMCR8 complex, a complex that has guanine nucleotide exchange factor (GEF) activity and regulates autophagy. In the complex, C9orf72 and SMCR8 probably constitute the catalytic subunits that promote the exchange of GDP to GTP, converting inactive GDP-bound RAB8A and RAB39B into their active GTP-bound form, thereby promoting autophagosome maturation. The C9orf72-SMCR8 complex also acts as a regulator of autophagy initiation by interacting with the ATG1/ULK1 kinase complex and modulating its protein kinase activity. It positively regulates initiation of autophagy by regulating the RAB1A-dependent trafficking of the ATG1/ULK1 kinase complex to the phagophore which leads to autophagosome formation. And acts as a regulator of mTORC1 signaling by promoting phosphorylation of mTORC1 substrates. C9orf72 may be involved in regulating the maturation of phagosomes to lysosomes. It regulates actin dynamics in motor neurons by inhibiting the GTP-binding activity of ARF6, leading to ARF6 inactivation. This reduces the activity of the LIMK1 and LIMK2 kinases which are responsible for phosphorylation and inactivation of cofilin, leading to cofilin activation. It positively regulates axon extension and axon growth cone size in spinal motor neurons. C9orf72 plays a role within the hematopoietic system in restricting inflammation and the development of autoimmunity. 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. Diseases associated with C9orf72 include Frontotemporal Dementia And/Or Amyotrophic Lateral Sclerosis 1 and Huntington Disease-Like Syndrome Due To C9orf72 Expansions. Anti-C9orf72 Antibody is useful for researchers interested in Neuroscience Research.
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基因ID
- 203228
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NCBI登录号
- NP_001242983
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UniProt
- Q96LT7
抗原
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