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Dokumenttyp:
journal article 
Autor(en):
Schludi, Martin H; May, Stephanie; Grässer, Friedrich A; Rentzsch, Kristin; Kremmer, Elisabeth; Küpper, Clemens; Klopstock, Thomas; German Consortium for Frontotemporal Lobar Degeneration; Bavarian Brain Banking Alliance; Arzberger, Thomas; Edbauer, Dieter; Danek, Adrian; Diehl-Schmid, Janine; Fassbender, Klaus; Förstl, Hans; Kornhuber, Johannes; Otto, Markus; Ceballos-Baumann, Andres; Dieterich, Marianne; Feuerecker, Regina; Giese, Armin; Klünemann, Hans; Kurz, Alexander; Levin, Johannes; Loren...    »
 
Titel:
Distribution of dipeptide repeat proteins in cellular models and C9orf72 mutation cases suggests link to transcriptional silencing. 
Abstract:
A massive expansion of a GGGGCC repeat upstream of the C9orf72 coding region is the most common known cause of amyotrophic lateral sclerosis and frontotemporal dementia. Despite its intronic localization and lack of a canonical start codon, both strands are translated into aggregating dipeptide repeat (DPR) proteins: poly-GA, poly-GP, poly-GR, poly-PR and poly-PA. To address conflicting findings on the predominant toxicity of the different DPR species in model systems, we compared the expression...    »
 
Zeitschriftentitel:
Acta Neuropathol (Berl) 
Jahr:
2015 
Band / Volume:
130 
Heft / Issue:
Seitenangaben Beitrag:
537-55 
Sprache:
eng 
Print-ISSN:
0001-6322 
TUM Einrichtung:
Klinik und Poliklinik für Psychiatrie und Psychotherapie