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外周髓鞘蛋白-22抗體品牌 英文名稱  Anti-PMP22

中文名稱  外周髓鞘蛋白-22抗體品牌 

別    名 GAS3; CMT1A; CMT1E; DSS; GAS-3; Growth Arrest Specific 3; Growth arrest-specific protein 3; HMSNIA; HNPP; MGC20769; Peripheral Myelin Protein 22; PMP-22; PMP22; PMP22_HUMAN; Sp110; Trembler.

產(chǎn)品屬性：

濃    度  1mg/1ml

規(guī) 格  0.1ml/100μg 0.2ml/200μg

抗體來源  Rabbit

克隆類型   polyclonal

交叉反應(yīng)   Human, Mouse, Rat

產(chǎn)品類型   一抗  

研究領(lǐng)域    免疫學(xué) 神經(jīng)生物學(xué) 糖蛋白

蛋白分子量  predicted molecular weight: 22kDa 

性    狀   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human PMP-22 C-terminus

亞    型  IgG

純化方法   affinity purified by Protein A

儲(chǔ) 存 液   0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide

外周髓鞘蛋白-22抗體品牌 產(chǎn)品應(yīng)用   WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:100-500 IF=1:100-500

（石蠟切片需做抗原修復(fù)） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存條件  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

產(chǎn)品介紹 PMP22 is a 22 kDa glycoprotein expressed in the compact myelin of the peripheral nervous system. In the peripheral nervous system, PMP 22 is produced by myelinating Schwann cells and is coexpressed with the genes for myelin basic protein (MBP) during nerve development and regeneration. Alterations in the level of this protein cause several genetic human diseases. If the protein is duplicated, patients develop Charcot Marie Tooth disease. If one copy of the gene is deleted, they suffer from the inherited tendency to pressure palsies.

Function : Might be involved in growth regulation, and in myelinization in the peripheral nervous system.

Subcellular Location : Cell membrane; Multi-pass membrane protein.

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 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.

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.

Similarity : Belongs to the PMP-22/EMP/MP20 family.

Database links : UniProtKB/Swiss-Prot: Q01453.1

神經(jīng)生物學(xué)相關(guān)蛋白（Neurobiology）

外周髓鞘蛋白22（PMP22）是一種糖蛋白，在外周中的致密肌纖維素中表達(dá)。它由髓鞘雪旺氏細(xì)胞產(chǎn)生，并在神經(jīng)發(fā)育和再生過程中與MBP和Po蛋白共同表達(dá)。該蛋白表達(dá)水平變化可引起幾種人類遺傳性疾病，如果該蛋白增多，則會(huì)發(fā)生Charcot-Marie-Tooth疾病，如果缺失，則易患壓力的遺傳傾向。

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