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外周髓鞘蛋白-22抗体Ys-0235R
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品牌名称：$brandModel.Title型号：Ys-0235R 原产地：中国大陆 更新时间：2026/11/10 8:31:36
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详细内容
产品编号Ys-0235R
英文名称PMP22
中文名称外周髓鞘蛋白-22抗体
别 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.
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Mouse, Rat, (predicted: Human, )
产品应用WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量22kDa
细胞定位细胞膜
性 状Liquid
浓 度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human PMP22: 101-160/160
亚 型IgG
纯化方法affinity purified by Protein A
缓 冲 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMedPubMed
产品介绍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 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 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.
SWISS:
Q01453
Gene ID:
5376
热门标签：外周髓鞘蛋白-22抗体,雅吉抗体
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