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| 规格： | 50ul | 产品价格： | ￥1180.0 |
|---|---|---|---|
| 规格： | 100ul | 产品价格： | ￥1980.0 |
| 规格： | 200ul | 产品价格： | ￥2800.0 |
| 产品编号 | bs-17249R |
| 英文名称 | MTMR13 Rabbit pAb |
| 中文名称 | 肌微管蛋白MTMR13抗体 |
| 英文别名 | SBF2; CMT4B2; KIAA1766; MTMR13; Myotubularin-related protein 13; SET-binding factor 2. |
| 产品应用 | IHC-P=1:100-500, IHC-F=1:100-500, ICC/IF=1:100-500, IF=1:100-500, ELISA=1:5000-10000 Not yet tested in other applications. |
| 交叉反应 | (Human, Mouse, Rat, Horse) |
| 抗体来源 | Rabbit |
| 免疫原 | KLH conjugated synthetic peptide derived from human MTMR13 |
| 亚型 | IgG |
| 性状 | Liquid |
| 纯化方法 | affinity purified by Protein A |
| 克隆类型 | Polyclonal |
| 理论分子量 | 208 kDa |
| 浓度 | 1mg/ml |
| 储存液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 研究领域 | Neuroscience > Neurology process > Neurodegenerative disease Signal Transduction > Signaling Pathway > G Protein Signaling > Small G Proteins > Regulators |
| 亚细胞定位 | Cell Membrane and Cytoplasmic. Peripheral membrane protein. |
| 功能 | Defects in SBF2 are the cause of Charcot-Marie-Tooth disease type 4B2 (CMT4B2). CMT4B2 is a recessive 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 and primary peripheral axonal neuropathy. Demyelinating CMT neuropathies 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. By convention, autosomal recessive forms of demyelinating Charcot-Marie-Tooth disease are designated CMT4. CMT4B2 is characterized by abnormal folding of myelin sheaths. CMT4B2 is a recessive 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 and primary peripheral axonal neuropathy. Demyelinating CMT neuropathies 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. By convention, autosomal recessive forms of demyelinating Charcot-Marie-Tooth disease are designated CMT4. CMT4B2 is characterized by abnormal folding of myelin sheaths. |
| 保存条件 | Shipped at 4℃. Store at -20℃ 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. |
| 背景资料 | This gene encodes a pseudophosphatase and member of the myotubularin-related protein family. This gene maps within the CMT4B2 candidate region of chromosome 11p15 and mutations in this gene have been associated with Charcot-Marie-Tooth Disease, type 4B2. [provided by RefSeq, Jul 2008] |
| 应用 | 推荐稀释比例 |
| {IHC-P} | {1:100-500} |
| {IHC-F} | {1:100-500} |
| {ICC/IF} | {1:100-500} |
| {IF} | {1:100-500} |
| {ELISA} | {1:5000-10000} |
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