Anti-Myelin Protein Zero抗体(ab64685)

概述

  • 产品名称Anti-Myelin Protein Zero抗体
    参阅全部 Myelin Protein Zero 一抗
  • 描述
    兔多克隆抗体to Myelin Protein Zero
  • 特异性This antibody is specific for the extracellular domain of Myelin Protein Zero.
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Mouse
  • 免疫原

    Synthetic peptide made against the extracellular domain of Myelin Protein Zero (183 amino acid N-terminal region) expressed in E. coli.

  • 阳性对照
    • Mouse sciatic nerve extract.

性能

应用

Our Abpromise guarantee covers the use of ab64685 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
WB 1/500. Detects a band of approximately 30 kDa (predicted molecular weight: 28 kDa).

靶标

  • 功能Creation of an extracellular membrane face which guides the wrapping process and ultimately compacts adjacent lamellae.
  • 组织特异性Found only in peripheral nervous system Schwann cells.
  • 疾病相关Defects in MPZ are the cause of Charcot-Marie-Tooth disease type 1B (CMT1B) [MIM:118200]. CMT1B 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. Neuropathies of the CMT1 group 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.
    Defects in MPZ are the cause of Charcot-Marie-Tooth disease type 2I (CMT2I) [MIM:607677]. CMT2I 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. Neuropathies of the CMT2 group 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. CMT2I is characterized by late onset (range 47 to 60 years).
    Defects in MPZ are the cause of Charcot-Marie-Tooth disease type 2J (CMT2J) [MIM:607736]. CMT2J is a form of Charcot-Marie-Tooth disease characterized by the association of axonal peripheral neuropathy with hearing loss and pupillary abnormalities such as Adie pupil. Inheritance is autosomal dominant.
    Defects in MPZ are the cause of Adie pupil (ADIEP) [MIM:103100]. A stationary, benign disorder characterized by tonic, sluggishly reacting pupil and hypoactive or absent tendon reflexes. Adie pupil is a characteristic of Charcot-Marie-Tooth disease type 2J.
    Defects in MPZ may be the cause of Charcot-Marie-Tooth disease dominant intermediate type D (CMTDID) [MIM:607791]. CMTDID is a form of Charcot-Marie-Tooth disease characterized by features intermediate between demyelinating and axonal peripheral neuropathies, and motor median nerve conduction velocities ranging from 25 to 45 m/sec.
    Defects in MPZ are a cause of Dejerine-Sottas syndrome (DSS) [MIM:145900]; also known as Dejerine-Sottas neuropathy (DSN) or hereditary motor and sensory neuropathy III (HMSN3). DSS is a severe degenerating neuropathy of the demyelinating Charcot-Marie-Tooth disease category, with onset by age 2 years. DSS is characterized by motor and sensory neuropathy with very slow nerve conduction velocities, increased cerebrospinal fluid protein concentrations, hypertrophic nerve changes, delayed age of walking as well as areflexia. There are both autosomal dominant and autosomal recessive forms of Dejerine-Sottas syndrome.
    Defects in MPZ are a cause of congenital hypomyelination neuropathy (CHN) [MIM:605253]. CHN is characterized clinically by early onset of hypotonia, areflexia, distal muscle weakness, and very slow nerve conduction velocities.
    Defects in MPZ are a cause of Roussy-Levy syndrome (ROULS) [MIM:180800]; also known as Roussy-Levy hereditary areflexic dystasia. This autosomal dominant disorder resembles Charcot-Marie-Tooth disease type 1 in that it presents with foot deformity, weakness and atrophy of distal limb muscles, especially the peronei, and absent tendon reflexes. The phenotype differs, however, in that it includes static tremor of the upper limbs and gait ataxia.
  • 序列相似性Belongs to the myelin P0 protein family.
    Contains 1 Ig-like V-type (immunoglobulin-like) domain.
  • 翻译后修饰N-glycosylated; contains sulfate-substituted glycan.
  • 细胞定位Membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Charcot Marie Tooth neuropathy 1B antibody
    • CHM antibody
    • CMT1 antibody
    • CMT1B antibody
    • CMT2I antibody
    • CMT2J antibody
    • CMT4E antibody
    • CMTDI3 antibody
    • CMTDID antibody
    • DSS antibody
    • HMSNIB antibody
    • MPP antibody
    • MPZ antibody
    • Myelin peripheral protein antibody
    • Myelin protein P0 antibody
    • Myelin protein zero antibody
    • MYP0_HUMAN antibody
    • P0 antibody
    see all

Anti-Myelin Protein Zero antibody 图像

  • Anti-Myelin Protein Zero antibody (ab64685) at 1/500 dilution + Mouse sciatic nerve extract at 20 µg

    Secondary
    Goat anti-rabbit at 5000

    Predicted band size : 28 kDa
    Observed band size : 30 kDa (why is the actual band size different from the predicted?)

Anti-Myelin Protein Zero antibody (ab64685)参考文献

This product has been referenced in:
  • Murray LM  et al. Pre-symptomatic development of lower motor neuron connectivity in a mouse model of severe spinal muscular atrophy. Hum Mol Genet 19:420-33 (2010). WB ; Mouse . Read more (PubMed: 19884170) »

See 1 Publication for this product

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I can confirm that this product will interact with the extracellular domain of Myelin Protein Zero.

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