In HD animal models, clearing away
abnormal huntingtin deposits has led to improvements in symptoms, and Alfy plays an important role in cleanup.
Things go awry when these fragments are drawn toward the globs of
abnormal huntingtin, instead of being processed further.
Zeitlin says the study may also help researchers find new targets for drugs against Huntington's — such as compounds that block the clumping of
abnormal huntingtin.
Not exact matches
This
abnormal binding causes more VCP accumulation on the mitochondria,» Nerve cells with VCP - mutant
huntingtin interacting inside them became dysfunctional and self - destructed.
HD is caused by a mutation in the human HTT gene that results in an
abnormal expansion and misfolding of the corresponding
huntingtin protein.
When caspases chopped off small peptides from the mutant region of
huntingtin, they thought, the peptides accumulated into the
abnormal globs seen in the neurons of Huntington's victims.
Huntington's disease is caused by the
abnormal repetition of a specific DNA sequence at the tail end of the
huntingtin gene.
The Max Planck scientists could also show that a toxic protein related to the
abnormal, aggregate - forming protein «
huntingtin» of patients with the neurodegenerative Huntington's disease is efficiently destroyed by the newly identified pathway.
R6 / 2 mice have been genetically altered with an
abnormal gene that makes them produce a harmful fragment of the mutant
huntingtin protein
The mutation causing HD is an
abnormal polyglutamine stretch in
huntingtin.
Huntington's Disease (HD) is an autosomal dominant inherited disease characterized by an
abnormal and unstable expansion in the number of CAG trinucleotide repeats in exon 1 of the
huntingtin gene (Group HsDCR, 1993).