|Place of birth||Elmshorn, Germany|
|Date of birth||29.08.1979|
|since 2007||PhD in the field of neuronal protein degradation with the subject "C. elegans as a model system to analyse Ataxin-3/ATX-3-dependent neurodegeneration". The work is carried out in the lab of Dr. Thorsten Hoppe at the Center for Molecular Neurobiology Hamburg (ZMNH), University of Hamburg|
|since 2007||Postgraduate studies in molecular biology at the University of Hamburg|
|2005/2006||Diplomatheses in the field of neurobiology with the subject "Identification of interaction partners and functions of the protein ATX-3/Ataxin-3 within the ubiquitin proteasome system in C.elegans". The work was carried out in the lab of Dr. Thorsten Hoppe at the Center for Molecular Neurobiology Hamburg (ZMNH), University of Hamburg. Grade: "sehr gut" (highest possible grade)|
|2000-2005||Study of biology at the University of Hamburg. Main subject: Human biology. Minor subjects: applied botany and cultural anthropology|
Molecular Mechanisms of Spinocerebellar Ataxia Type 3 (SCA3)
The neurodegenerative disease spinocerebellar ataxia type 3 (SCA3) is the most common dominantly inherited ataxia. SCA3 belongs to a group of at least nine polyQ (polyglutamine) ailments.
These polyQ disorders are based on the pathological accumulation of the amino acid glutamine within the polypeptide, which causes mis- folding and subsequent accumulation of the insoluble disease protein in neurons. The pathogenesis of SCA3 is slowly progressive with a late onset of dysfunctions impairing postural balance and coor- dination of all body movements, including gait, speech and fine movements of the hands.
Mutations in the ataxin-3 gene are in most cases responsible for inherited forms of SCA3. The Ataxin-3 protein is a ubiquitin-specific protease; however, its precise role throughout the ubiquitin- proteasome system (UPS) is not clear at present. In this context it is noteworthy that several different neuro- degenerative diseases, such as SCA3, are associated with defects within the UPS.
The aim of my PhD thesis is to gain insights into the molecular mechanisms that underly the SCA3 disease using Caenorhabditis elegans as a model organism. I will combine biochemical and genetic approaches to identify new factors involved in the pathogenesis of this polyQ disease. Furthermore, functional in vitro and in vivo studies may help to elucidate the physiological role of the Ataxin-3 protein. A prime objective is to establish a disease model for SCA3 in C.elegans by per- forming complementation studies with human Ataxin-3 and the C.ele- gans Homolog ATX-3 to understand the interplay between Ataxin-3 mutations and protein aggregation.