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    Rabbit Anti-MTMR13/Gold Conjugated antibody (bs-17249R-Gold)
    訂購熱線(xiàn):400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術(shù)支持:techsupport@bioss.com.cn
    說(shuō) 明 書(shū): 100ul(10nm  15nm  35nm
    100ul/2980.00元
    大包裝/詢(xún)價(jià)
    產(chǎn)品編號 bs-17249R-Gold
    英文名稱(chēng) Rabbit Anti-MTMR13/Gold Conjugated antibody
    中文名稱(chēng) 膠體金標記的肌微管蛋白MTMR13抗體
    別    名 SBF2; CMT4B2; KIAA1766; MTMR13; Myotubularin-related protein 13; SET-binding factor 2.  
    規格價(jià)格 100ul/2980元 購買(mǎi)        大包裝/詢(xún)價(jià)
    說(shuō) 明 書(shū) 100ul(10nm  15nm  35nm
    研究領(lǐng)域 細胞生物  神經(jīng)生物學(xué)  信號轉導  G蛋白信號  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    交叉反應 (predicted: Human, Mouse, Rat, Horse, )
    產(chǎn)品應用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 208kDa
    性    狀 Lyophilized or Liquid
    濃    度 0.4mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human MTMR13
    亞    型 IgG
    純化方法 affinity purified by Protein A
    儲 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
    保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
    產(chǎn)品介紹 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 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.

    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.
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