概述

  • 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
  • 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 conjugated to KLH derived from within residues 300 to the C-terminus of Human GDAP1.

  • 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.

性能

  • 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
    Note: 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...
  • 纯度Immunogen affinity purified
  • 克隆多克隆
  • 同种型IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab100905 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
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

  • 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
  • 数据库链接
  • 别名
    • Charcot Marie Tooth neuropathy 4A antibody
    • CMT4 antibody
    • CMT4A antibody
    • CMTRIA antibody
    • Ganglioside induced differentiation associated protein 1 antibody
    • Ganglioside-induced differentiation-associated protein 1 antibody
    • GDAP1 antibody
    • GDAP1_HUMAN antibody
    see all

Anti-GDAP1 antibody 图像

  • 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
    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.

Anti-GDAP1 antibody (ab100905)参考文献

ab100905 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab100905.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"