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    Mouse Anti-PLP1/PE-Cy7 Conjugated antibody (bsm-51197M-PE-Cy7)
    訂購熱線:400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術支持:techsupport@bioss.com.cn
    說 明 書: 100ul  
    100ul/2980.00元
    大包裝/詢價
    產品編號 bsm-51197M-PE-Cy7
    英文名稱 Mouse Anti-PLP1/PE-Cy7 Conjugated antibody
    中文名稱 PE-Cy7標記的小鼠抗髓磷酯髓鞘蛋白1單克隆抗體
    別    名 Proteolipid Protein 1; Lipophilin; PLP; Spastic Paraplegia 2, Uncomplicated; Major Myelin Proteolipid Protein; Pelizaeus-Merzbacher Disease; Myelin Proteolipid Protein; PLP/DM20; GPM6C; HLD1; MMPL; SPG2; PMD; MYPR_RAT;   
    規格價格 100ul/2980元 購買        大包裝/詢價
    說 明 書 100ul  
    研究領域 神經生物學  細胞粘附分子  
    抗體來源 Mouse
    克隆類型 Monoclonal
    克 隆 號 4E7
    交叉反應 (predicted: Human, Rat, )
    產品應用
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 30kDa
    性    狀 Lyophilized or Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from rat PLP1
    亞    型 IgM
    純化方法 affinity purified by Protein AGL
    儲 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.05M PB, pH 7.5.
    保存條件 Store at -20 °C 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 antibody the antibody is stable for at least two weeks at 2-4 °C.
    產品介紹 background:
    PLP is a major constituent of myelin. The two isoforms of the myelin proteolipid protein, PLP and DM20, are very hydrophobic integral membrane proteins that account for about half of the protein content of adult CNS myelin. A mutation in the gene which encodes PLP is linked to Pelizaeus-Merzbacher disease (PMD), a chronic infantile type of diffuse cerebral sclerosis. The gene which encodes PLP maps to human chromosome Xq22. The glycoprotein zero (also designated P-zero or myelin peripheral protein) is the primary structural protein of peripheral myelin, and accounts for more than 50% of the protein present in the peripheral nerve sheath. Zero is an integral membrane glycoprotein. Expression of zero is restricted to Schwann cells. The gene which encodes zero maps to human chromosome 1q22. PMP22 (peripheral myelin protein 22) is a growth-regulated membrane protein which is expressed by Schwann cells and is localized primarily in compact peripheral nervous system myelin. The gene which encodes PMP22 maps to human chromosome 17p11.2.

    Function:
    This is the major myelin protein from the central nervous system. It plays an important role in the formation or maintenance of the multilamellar structure of myelin.

    Subunit:
    Belongs to the myelin proteolipid protein family.

    Subcellular Location:
    Membrane; Multi-pass membrane protein.

    DISEASE:
    Defects in PLP1 are the cause of leukodystrophy hypomyelinating type 1 (HLD1) [MIM:312080]; also known as Pelizaeus-Merzbacher disease. HLD1 is an X-linked recessive dysmyelinating disorder of the central nervous system in which myelin is not formed properly. It is characterized clinically by nystagmus, spastic quadriplegia, ataxia, and developmental delay.
    Defects in PLP1 are the cause of spastic paraplegia X-linked type 2 (SPG2) [MIM:312920]. SPG2 is characterized by spastic gait and hyperreflexia. In some patients, complicating features include nystagmus, dysarthria, sensory disturbance, mental retardation, optic atrophy.

    Similarity:
    Belongs to the myelin proteolipid protein family.

    Database links:

    Entrez Gene: 281410 Cow

    Entrez Gene: 481002 Dog

    Entrez Gene: 5354 Human

    Entrez Gene: 18823 Mouse

    Entrez Gene: 24943 Rat

    Omim: 300401 Human

    SwissProt: P04116 Cow

    SwissProt: P23294 Dog

    SwissProt: P60201 Human

    SwissProt: P60202 Mouse

    SwissProt: P60203 Rat

    Unigene: 49691 Cow

    Unigene: 1787 Human

    Unigene: 1268 Mouse

    Unigene: 4550 Rat



    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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