| 產(chǎn)品編號(hào) | bsm-54072R |
| 英文名稱 | PMP22 Recombinant Rabbit mAb |
| 中文名稱 | 外周髓鞘蛋白-22重組兔單抗 |
| 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. |
| 研究領(lǐng)域 | 免疫學(xué) 神經(jīng)生物學(xué) 糖蛋白 |
| 抗體來源 | Rabbit |
| 克隆類型 | Recombinant |
| 克 隆 號(hào) | 1A11 |
| 交叉反應(yīng) | Human,Mouse,Rat |
| 產(chǎn)品應(yīng)用 | WB=1:300-1000,IHC-P=1:100-500,IHC-F=1:50-200,IF=1:50-100,Flow-Cyt=1:20-100,ICC/IF=1:50-100
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 22 kDa |
| 檢測(cè)分子量 | |
| 細(xì)胞定位 | 細(xì)胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | Recombinant human PMP22 protein(Full length) |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Store at -20℃ 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 antib |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 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 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 Database links: Entrez Gene: 5376 Human Omim: 601097 Human SwissProt: Q01453 Human Unigene: 372031 Human Unigene: 1476 Rat 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，在外周神經(jīng)系統(tǒng)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，則病人會(huì)發(fā)生Charcot-Marie-Tooth疾病，如果缺失，則易患?jí)毫β楸缘倪z傳傾向。 |
| 產(chǎn)品圖片 |
Western blot analysis of PMP22 on PC-12 cell lysates. Proteins were transferred to a PVDF membrane and blocked with 5% BSA in PBS for 1 hour at room temperature. The primary antibody (bsm-54072R, 1/500) was used in 5% BSA at room temperature for 2 hours. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1:5,000 dilution was used for 1 hour at room temperature.
Immunohistochemical analysis of paraffin-embedded human pancreas tissue with Rabbit anti-PMP22 antibody (bsm-54072R) at 1/500 dilution.
The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (bsm-54072R) at 1/500 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.
Immunohistochemical analysis of paraffin-embedded rat brain tissue with Rabbit anti-PMP22 antibody (bsm-54072R) at 1/500 dilution.
The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (bsm-54072R) at 1/500 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.
ICC staining of PMP22 in N2A cells (green). Formalin fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 10 minutes at room temperature and blocked with 1% Blocker BSA for 15 minutes at room temperature. Cells were probed with the primary antibody (bsm-54072R, 1/50) for 1 hour at room temperature, washed with PBS. Alexa Fluor?488 Goat anti-Rabbit IgG was used as the secondary antibody at 1/1,000 dilution. The nuclear counter stain is DAPI (blue).
Flow cytometric analysis of PMP22 was done on SH-SY5Y cells. The cells were fixed, permeabilized and stained with the primary antibody (bsm-54072R, 1/50) (red). After incubation of the primary antibody at room temperature for an hour, the cells were stained with a Alexa Fluor 488-conjugated Goat anti-Rabbit IgG Secondary antibody at 1/1,000 dilution for 30 minutes.Unlabelled sample was used as a control (cells without incubation with primary antibody; black).
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