Year: 2009
Type: other
Abstract: present in vivo, using a SCA1 mouse model.Furthermore, we investigated whether the localization and dynamics of three allegedly ataxin-1 interacting proteins, LANP, PQBP-1 and RBM17, are altered when they are co-expressed with mutant ataxin-1.In chapter 4, we search for a common pathway of ataxin-1 and PKCγ (the protein mutated in SCA14) by studying interactions between the two proteins.We also study the effects of PKCγ on ataxin-1 nuclear body formation and dynamics, and vice versa the effects of mutant ataxin-1 on the distribution of PKCγ in SCA1 mouse.In chapter 5, we discuss the different molecular and cellular levels at which mutant ataxin-1 can initiate toxicity.Here, we start in the nucleus and travel towards the cell periphery to discuss normal and toxic functions of ataxin-1 in each cellular compartment.PolyQ proteins are being degraded by proteases like the proteasome.In the last part of this thesis, chapter 6, we mimic generation of polyQ fragments by the proteasome and examine whether these fragments are degradation-resistant and sufficient to initiate aggregation.HY and Orr HT (1993) Evidence for a mechanism predisposing to intergenerational CAG repeat instability in spinocerebellar ataxia type I.
Author: Hilde A. Krol
Language: English
Subfield: Cellular and Molecular Neuroscience
Field: Neuroscience
Domain: Life Sciences
Open Access status: green
OpenAlex ID: W2120749876