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merck millipore,默克密理博,MAB2265,Anti-Peripheral Myelin Protein 22 (PMP22) Antibody, clone CF1
产品名称：Anti-Peripheral Myelin Protein 22 (PMP22) Antibody, clone CF1
产品型号：MAB2265
Anti-Peripheral Myelin Protein 22 (PMP22) Antibody, clone CF1 is an antibody against Peripheral Myelin Protein 22 (PMP22) for use in IH.
merck millipore,默克密理博,MAB2265,Anti-Peripheral Myelin Protein 22 (PMP22) Antibody, clone CF1
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 H IHC M Purified Monoclonal Antibody 描述 产品目录编号 MAB2265 描述 Anti-Peripheral Myelin Protein 22 (PMP22) Antibody, clone CF1 Alternate Names - growth arrest-specific 3
- peripheral myelin protein 22
- Growth arrest-specific protein 3
背景信息 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). 产品信息 格式 Purified 控制 - Human normal cortex tissue
演示 Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. 应用 应用 Anti-Peripheral Myelin Protein 22 (PMP22) Antibody, clone CF1 is an antibody against Peripheral Myelin Protein 22 (PMP22) for use in IH. 主要应用 - Immunohistochemistry
生物信息 免疫原品种 Human PMP22 cDNA boosted with 13-mer peptide of the second extracellular domain of PMP22 表位 Unknown 克隆 CF1 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Mouse 特异性 This antibody recognizes peripheral myelin protein 22 (PMP22) 同种型 IgG1κ 品种反应性 Human Species Reactivity Note Demonstrated to react with human. 抗体类型 Monoclonal Antibody Entrez基因编号 - NP_000295
Entrez基因汇总 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]. 基因符号 - Sp110
- PMP22
- GAS3
- CMT1A
- PMP-22
- DSS
- CMT1E
- GAS-3
- HMSNIA
- HNPP
纯化方法 Protein G UniProt编号 - Q01453
UniProt汇总 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.41
Defects 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.45
Defects 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.产品使用声明 质量保证 Evaluated by Immunohistochemistry in human normal cortex tissue.
Immunohistochemistry Analysis: 1:500 dilution of this antibody detected PMP22 in human normal cortex tissue.使用声明 - 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.
储存和货运信息 存储条件 Stable for 1 year at 2-8°C from date of receipt. 包装信息 数量 100 µg
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