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Huntington disease
Huntington disease (HD) is an autosomal-dominant neurodegenerative disorder that primarily affects medium spiny striatal neurons (MSN). The symptoms are choreiform, involuntary movements, personality changes and dementia. HD is caused by a CAG repeat expansion in the IT15 gene, which results in a long stretch of polyglutamine (polyQ) close to the amino-terminus of the HD protein huntingtin (Htt). Mutant Htt (mHtt) has effects both in the cytoplasm and in the nucleus. Full-length huntingtin is cleaved by proteases in the cytoplasm, leading to the formation of cytoplasmic and neuritic aggregates. mHtt also alters vesicular transport and recycling, causes cytosolic and mitochondrial Ca2+ overload, triggers endoplasmic reticulum stress through proteasomal dysfunction, and impairs autophagy function, increasing neuronal death susceptibility. N-terminal fragments containing the polyQ strech translocate to the nucleus where they impair transcription and induce neuronal death.
产品列表
    货号
    产品
    反应
    应用
  • CLCA Rabbit pAb
    Human,Mouse,Rat
    WB
  • CLCB Rabbit pAb
    Human,Mouse,Rat
    WB
  • Clathrin Heavy Chain 1 (PT0067R) PT® Rabbit mAb
    Human,Mouse,Rat,
    WB,IHC,IF,ELISA
  • CLH1 Rabbit pAb
    Human,Rat,Mouse,
    WB,ELISA
  • CLH2 Rabbit pAb
    Human
    WB
  • HIP1 Rabbit pAb
    Human,Mouse
    WB
  • AP2A2 Rabbit pAb
    Human,Mouse,Rat
    WB
  • AP2A1 Rabbit pAb
    Human,Mouse
    WB
  • AP2B1 Rabbit pAb
    Human,Mouse,Rat
    WB
  • AP2M1 Rabbit pAb
    Human,Mouse,Rat
    WB,ELISA
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  • 3
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  • 68