IndraLab

Statements

HTT activates ATXN3. 5 / 5
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"And, indeed, similar genome engineering approaches have been shown protective in preclinical studies in, a Htt lowering approach in HD, and CAG expansion reduction approaches in SCA3."
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"However, blocking of the autophagic degradation exhibited no significant influence on the decline of Atx3 caused by Atx7 93Q -N172 or Htt 100Q -N90 (Supplemental Fig. 2B, D)."
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"In this study we screened a cohort of 21 Greek patients with HD phenocopy syndromes formutations causing HDL2, SCA17, SCA1, SCA2, SCA3, SCA8, SCA12 and DRPLA."
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"The same group demonstrated the late-onset transgenic model with ataxin-3 driven by huntingtin promoter."
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"Huntington’s disease (HD) and spinocerebellar ataxia type 3 (SCA3, also called Machado-Joseph disease) are caused by extensive polyglutamine (polyQ) expansion of the huntingtin or ataxin-3 protein [334]."
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