Dymeclin Antibody Blocking Peptide(bs-13037P)-500ug

Dymeclin Antibody Blocking Pep

tide(bs-13037P)-500ug
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  • ¥880
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  • bs-13037P
  • 2025年10月16日
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      500ug

    产品编号bs-13037P
    英文名称Dymeclin Antibody Blocking Peptide
    中文名称迪格弗-梅尔基奥尔-克劳森综合征相关蛋白封闭多肽
    英文别名DMC; Dyggve-Melchior-Clausen syndrome protein; DYM; FLJ20071; FLJ90130; SMC; DYM_HUMAN.
    纯化方法HPLC
    研究领域

    Developmental Biology > Organogenesis > Nervous system development

    Developmental Biology > Organogenesis > Skeletal development > Bone

    Signal Transduction > Protein Trafficking > Golgi Proteins

    亚基Interacts with GOLM1 and PPIB.
    亚细胞定位Cytoplasmic and Golgi Apparatus
    组织特异性Expressed in most embryo-fetal and adult tissues. Abundant in primary chondrocytes, osteoblasts, cerebellum, kidney, lung, stomach, heart, pancreas and fetal brain. Very low or no expression in the spleen, thymus, esophagus, bladder and thyroid gland.
    翻译后修饰Myristoylated in vitro; myristoylation is not essential for protein targeting to Golgi compartment.
    相似性Belongs to the dymeclin family.
    功能Necessary for correct organization of Golgi apparatus. Involved in bone development.
    保存条件Shipped at 4℃. Stored 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Dyggve-Melchior-Clausen syndrome (DMC), a rare autosomal recessive disorder, is characterized by microcephaly, short trunk dwarfism and sometime psychomotor retardation. Cutaneous cells of affected individuals show dilated rough endoplasmic reticulum and enlarged vacuoles. The Dyggve-Melchior-Clausen syndrome protein, also designated dymeclin, may play a role in proteoglycan metabolism and intracellular protein digestion. It is a widely expressed multi-pass membrane protein, detected primarily in chondrocytes and fetal brain tissue. Defects in dymeclin are also the cause of Smith-McCort dysplasis syndrome (SMC), which has characteristics identical to those of Dyggve-Melchior-Clausen syndrome.

     

     

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