肌微管蛋白MTMR13抗体
规格：1mg/1ml
英文名： MTMR13
别名： SBF2; CMT4B2; KIAA1766; MTMR13; Myotubularin-related protein 13; SET-binding factor 2.
分子量： 208kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human MTMR13
交叉反应：Human,
细胞定位：细胞浆 细胞膜
肌微管蛋白MTMR13抗体产品介绍：background: 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] Function: 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肌微管蛋白MTMR13抗体 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肌微管蛋白MTMR13抗体 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. Subcellular Location: Cell Membrane and Cytoplasmic. Peripheral membrane protein. Database links: Entrez Gene: 81846 Human Omim: 607697 Human SwissProt: Q86WG5 Human Unigene: 577252 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
肌微管蛋白MTMR13抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=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.
研究领域：细胞生物 神经生物学 信号转导 G蛋白信号
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid