We report on the role of GGGGCC (G4C2) repeat in neurotoxicity. We show that only hnRNP-H binds directly to G4C2 and that only hnRNP-H co-localizes with 70% of G4C2 RNA foci detected in C9ORF72. We show that expanded G4C2 repeats are potentially neurotoxic and bind hnRNP-H and other RNA binding proteins. We propose that RNA toxicity and protein sequestration may disrupt RNA processing and contribute to neurodegeneration.
COBISS.SI-ID: 27328295
Orange is a machine learning and data mining suite for data analysis through Python scripting and visual programming. We report on the scripting part, which features interactive data analysis and component-based assembly of data mining procedures. In the selection and design of components, we focus on the flexibility of their reuse: our principal intention is to let the user write simple and clear scripts in Python, which build upon C++ implementations of computationally-intensive tasks. Orange is intended both for experienced users and programmers, as well as for students of data mining. In this research project we are using orange as an important part of advanced analysis and modeling.
COBISS.SI-ID: 10118740
We review the studies that integrated UV-induced cross-linked immunoprecipitation (CLIP) with other genome-wide methods to comprehensively characterise the function of diverse RBPs in the brain. We discuss the technical challenges of these studies and review the strategies that can be used to reliably identify the RNAs bound and regulated by an RBP. We conclude by highlighting how CLIP and related techniques have been instrumental in addressing the role of RBPs in neurologic diseases.