货号：MAB2265

品牌：Merck Millipore

规格：

目录价：￥4146.00

市场价格：￥3524.10

会员价格：￥3316.80

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Peripheral Myelin Protein 22 (PMP22) Antibody, clone CF1 | MAB2265 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Specificity: This antibody recognizes peripheral myelin protein 22 (PMP22) View All» Molecular Weight: 18 kDa calculated View All» Epitope: Unknown View All» Immunogen: Human PMP22 cDNA boosted with 13-mer peptide of the second extracellular domain of PMP22 View All» Clone: CF1 View All» Isotype: IgG1κ View All» Background Information: Peripheral myelin protein 22 (PMP22), a 160 amino acid glycoprotein, belongs to the claudin family of proteins. PMP22 is thought to have a critical role in external mesaxon formation during development as well as potential involvement in the progression of axon myelination. This glycoprotein has four hydrophobic domains a feature which gives rise to another potential role for PMP22 in the structure of peripheral nerve myelin. PMP22 has been observed in compact myelin found within the peripheral nerve of human adult, and has been noted as having a distribution similar to that of myelin Protein zero (PO). Defects in PMP-22 expression are causal to several hereditary demyelinating neuropathies including; inflammatory demyelinating polyneuropathy (IDP), hereditary neuropathy with liability to pressure palsies (HNPP), Charcot-Marie-Tooth disease Type 1A (CMT1A) and type 1E (CMT1E), and Dejerine-Sottas syndrome (DSS). View All» Species Reactivity: Human View All» Species Reactivity Note: Demonstrated to react with human. View All» Control: Human normal cortex tissue View All» Quality Assurance: Evaluated by Immunohistochemistry in human normal cortex tissue.Immunohistochemistry Analysis: 1:500 dilution of this antibody detected PMP22 in human normal cortex tissue. View All» Purification Method: Protein G View All» Presentation: Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. View All» Storage Conditions: Stable for 1 year at 2-8°C from date of receipt. View All» UniProt Number: Q01453 View All» Entrez Gene Number: NP_000295 View All» Gene Symbol:

• PMP22

• GAS3

• CMT1A

• PMP-22

• DSS

• CMT1E

• GAS-3

• HMSNIA

• HNPP

• Sp110
  View All» Alternate Names:
  • peripheral myelin protein 22

  • Growth arrest-specific protein 3

  • growth arrest-specific 3
    View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications: Immunohistochemistry View All» Entrez Gene Summary: This gene encodes an integral membrane protein that is a major component of myelin in the peripheral nervous system. Various mutations of this gene are causes of Charcot-Marie-Tooth disease Type IA, Dejerine-Sottas syndrome, and hereditary neuropathy with liability to pressure palsies. Alternative splicing of this gene results in three transcript variants that encode the same protein. [provided by RefSeq]. View All» UniProt Summary: FUNCTION: Might be involved in growth regulation, and in myelinization in the peripheral nervous system.SUBCELLULAR LOCATION: Membrane; Multi-pass membrane protein. INVOLVEMENT IN DISEASE: Defects in PMP22 are the cause of Charcot-Marie-Tooth disease type 1A (CMT1A) [MIM:118220]; also known as hereditary motor and sensory neuropathy IA. CMT1A 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. CMT1A inheritance is autosomal dominant.Defects in PMP22 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. Ref.10 Ref.11 Ref.14 Ref.15 Ref.18 Ref.19 Ref.20 Ref.21 Ref.24 Ref.25 Ref.26 Ref.28 Ref.31 Ref.33 Ref.37 Ref.41Defects in PMP22 are a cause of hereditary neuropathy with liability to pressure palsies (HNPP) [MIM:162500]; an autosomal dominant disorder characterized by transient episodes of decreased perception or peripheral nerve palsies after slight traction, compression or minor traumas. Ref.29 Ref.44 Ref.45Defects in PMP22 are the cause of Charcot-Marie-Tooth disease type 1E (CMT1E) [MIM:118300]; also known as Charcot-Marie-Tooth disease and deafness autosomal dominant. CMT1E is an autosomal dominant form of Charcot-Marie-Tooth disease characterized by the association of sensorineural hearing loss with peripheral demyelinating neuropathy.Defects in PMP22 may be a cause of inflammatory demyelinating polyneuropathy (IDP) [MIM:139393]. IDP is a putative autoimmune disorder presenting in an acute (AIDP) or chronic form (CIDP). The acute form is also known as Guillain-Barre syndrome. SEQUENCE SIMILARITIES: Belongs to the PMP-22/EMP/MP20 family. View All» Product Name: Anti-Peripheral Myelin Protein 22 (PMP22), clone CF1 View All» Concentration: 1.0 mg/mL View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 μg View All» Format: Purified View All» Host: Mouse View All»
    © 金山科研平台是专业的授权总代理区域代理经销平台。
    © 如需询价，请加客服QQ：1749072012 、客服微信：jinshanbio，或发送邮件到1749072012@qq.com
    © 平台为生命科学研究相关领域提供一站式耗材试剂仪器解决方案和采购服务，数据资源基于CC协议。
    © 本文地址：https://16ao.com/thread-130682.htm
    © Merck Millipore代理MAB2265 Anti-Peripheral Myelin Protein 22 (，产品报价联系微信jinshanbio