Anti-GDAP1抗体(ab100905)
Key features and details
- Rabbit polyclonal to GDAP1
- Suitable for: ICC/IF, WB
- Reacts with: Mouse, Rat, Human
- Isotype: IgG
概述
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产品名称
Anti-GDAP1抗体
参阅全部 GDAP1 一抗 -
描述
兔多克隆抗体to GDAP1 -
宿主
Rabbit -
经测试应用
适用于: ICC/IF, WBmore details -
种属反应性
与反应: Mouse, Rat, Human
预测可用于: Cow, Dog, Orangutan -
免疫原
Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.
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阳性对照
- This antibody gave a positive signal in the following tissue lysates: Human brain; Mouse brain; Mouse spinal cord; Rat brain; Rat spinal cord.
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常规说明
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle. -
存储溶液
pH: 7.40
Preservative: 0.02% Sodium azide
Constituent: PBS
Batches of this product that have a concentration < 1mg/ml may have BSA added as a stabilising agent. If you would like information about the formulation of a specific lot, please contact our scientific support team who will be happy to help. -
Concentration information loading...
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纯度
Immunogen affinity purified -
克隆
多克隆 -
同种型
IgG -
研究领域
相关产品
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Compatible Secondaries
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Isotype control
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Recombinant Protein
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab100905于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
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ICC/IF |
Use a concentration of 10 µg/ml.
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WB |
Use a concentration of 1 µg/ml. Detects a band of approximately 41 kDa (predicted molecular weight: 41 kDa).
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说明 |
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ICC/IF
Use a concentration of 10 µg/ml. |
WB
Use a concentration of 1 µg/ml. Detects a band of approximately 41 kDa (predicted molecular weight: 41 kDa). |
靶标
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功能
May function in a signal transduction pathway responsible for ganglioside-induced neurite differentiation. May also have a role in protecting myelin membranes against free radical-mediated damage. -
组织特异性
Highly expressed in whole brain and spinal cord. Predominant expression in central tissues of the nervous system not only in neurons but also in Schwann cells. -
疾病相关
Defects in GDAP1 are the cause of Charcot-Marie-Tooth disease type 4A (CMT4A) [MIM:214400]. CMT4A is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Demyelinating CMT neuropathies are characterized by severely reduced nerve conduction velocities (less than 38 m/sec), segmental demyelination and remyelination with onion bulb formations on nerve biopsy, slowly progressive distal muscle atrophy and weakness, absent deep tendon reflexes, and hollow feet. Autosomal recessive forms of demyelinating Charcot-Marie-Tooth disease are by convention designated CMT4. CMT4A is a severe form characterized by early age of onset and rapid progression leading to inability to walk in late childhood or adolescence.
Defects in GDAP1 are the cause of Charcot-Marie-Tooth disease axonal recessive with vocal cord paresis (CMT2RV) [MIM:607706]. CMT2RV is a form of Charcot-Marie-Tooth disease characterized by the association of axonal neuropathy with vocal cord paresis.
Defects in GDAP1 are the cause of Charcot-Marie-Tooth disease type 2K (CMT2K) [MIM:607831]. CMT2K is an axonal form of Charcot-Marie-Tooth disease. Axonal CMT neuropathies are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. CMT2K onset is in early childhood (younger than 3 years). This phenotype is characterized by foot deformities, kyphoscoliosis, distal limb muscle weakness and atrophy, areflexia, and diminished sensation in the lower limbs. Weakness in the upper limbs is observed in the first decade, with clawing of the fingers. Inheritance can be autosomal dominant or recessive.
Defects in GDAP1 are the cause of Charcot-Marie-Tooth disease recessive intermediate type A (CMTRIA) [MIM:608340]. CMTRIA is a form of Charcot-Marie-Tooth disease characterized by clinical and pathologic features intermediate between demyelinating and axonal peripheral neuropathies, and motor median nerve conduction velocities ranging from 25 to 45 m/sec. -
序列相似性
Belongs to the GST superfamily.
Contains 1 GST C-terminal domain.
Contains 1 GST N-terminal domain. -
细胞定位
Cytoplasm. - Information by UniProt
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数据库链接
- Entrez Gene: 613472 Cow
- Entrez Gene: 54332 Human
- Entrez Gene: 14545 Mouse
- Entrez Gene: 312890 Rat
- Omim: 606598 Human
- SwissProt: A6QQZ0 Cow
- SwissProt: Q8TB36 Human
- SwissProt: O88741 Mouse
see all -
别名
- Charcot Marie Tooth neuropathy 4A antibody
- CMT4 antibody
- CMT4A antibody
see all
图片
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All lanes : Anti-GDAP1 antibody (ab100905) at 1 µg/ml
Lane 1 : Human brain tissue lysate - total protein (ab29466)
Lane 2 : Brain (Mouse) Tissue Lysate
Lane 3 : Spinal Cord (Mouse) Tissue Lysate
Lane 4 : Brain (Rat) Tissue Lysate
Lane 5 : Spinal Cord (Rat) Tissue Lysate
Lysates/proteins at 10 µg per lane.
Secondary
All lanes : Goat Anti-Rabbit IgG H&L (HRP) preadsorbed (ab97080) at 1/5000 dilution
Developed using the ECL technique.
Performed under reducing conditions.
Predicted band size: 41 kDa
Observed band size: 41 kDa
Additional bands at: 55 kDa. We are unsure as to the identity of these extra bands.
Exposure time: 30 seconds -
ICC/IF image of ab100905 stained SKNSH cells. The cells were 4% PFA fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab100905, 10µg/ml) overnight at +4°C. The secondary antibody (green) was ab96899, DyLight® 488 goat anti-rabbit IgG (H+L) used at a 1/250 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.
实验方案
数据表及文件
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SDS download
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Datasheet download
文献 (2)
ab100905 被引用在 2 文献中.
- Duan C et al. Drp1 regulates mitochondrial dysfunction and dysregulated metabolism in ischemic injury via Clec16a-, BAX-, and GSH- pathways. Cell Death Dis 11:251 (2020). PubMed: 32312970
- Lapek JD et al. Defining Host Responses during Systemic Bacterial Infection through Construction of a Murine Organ Proteome Atlas. Cell Syst 6:579-592.e4 (2018). PubMed: 29778837