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SPG21 Antibody Blocking Peptid

e(bs-11785P)-500ug
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  • ¥880
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  • bs-11785P
  • 2025年10月16日
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      500ug

    产品编号bs-11785P
    英文名称SPG21 Antibody Blocking Peptide
    中文名称痉挛性截瘫相关蛋白21封闭多肽
    英文别名Acid cluster protein 33; ACP33; BM019; BM-019; GL010; MAST; Maspardin; Spastic paraplegia 21 autosomal recessive Mast syndrome protein; SPG21 antibody; SPG21_HUMAN.
    纯化方法HPLC
    研究领域

    Immunology > Adaptive Immunity > T Cells > Non-CD

    Neuroscience > Neurology process > Neurodegenerative disease

    亚基Interacts with CD4. Interacts with ALDH16A1.
    亚细胞定位Cytoplasm; cytosol. Membrane; peripheral membrane protein.
    组织特异性Expressed in all tissues tested, including heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas. Expressed in J.CaM1.6, HuT 78 and HeLa cell lines (at protein level).
    相似性Belongs to the AB hydrolase superfamily.
    功能Defects in SPG21 are the cause of Mast syndrome, an autosomal recessive hereditary spastic paraplegia with dementia and other CNS abnormalities (SPG21). Present at high frequency among the Old Order Amish. Subtle childhood abnormalities may be present, but the main features develop in early adulthood. The disease is slowly progressive, and cerebellar and extrapyramidal signs are also found in patients with advanced disease. Patients have a thin corpus callosum and white matter abnormalities. The protein encoded by this gene was identified by a two hybrid screen using CD4 as the bait. It binds to the hydrophobic C terminal amino acids of CD4 which are involved in repression of T cell activation. The interaction with CD4 is mediated by the noncatalytic alpha/beta hydrolase fold domain of this protein. It is thus proposed that this gene product modulates the stimulatory activity of CD4.
    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Maspardin is a 308 amino acid cytoplasmic protein that is widely expressed. Belonging to the AB hydrolase superfamily, Maspardin colocalizes with CD4 on endosomal/trans-Golgi network. It is thought that Maspardin may act as a negative regulatory factor in CD4-dependent T-cell activation. Defects in the gene encoding Maspardin are the result of hereditary spastic paraplegia autosomal recessive type 21 (also designated Mast syndrome), an autosomal recessive neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. The gene encoding Maspardin is encoded by human chromosome 15, which houses over 700 genes and comprises nearly 3% of the human genome.

     

     

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