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Transglutaminase 6 Is Colocalized and Interacts with Mutant Huntingtin in Huntington Disease Rodent Animal Models

Int J Mol Sci. 2021-08; 
Anja Schulze-Krebs, Fabio Canneva, Judith Stemick, Anne-Christine Plank, Julia Harrer, Gillian P Bates, Daniel Aeschlimann, Joan S Steffan, Stephan von Hörsten
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Mutant Libraries … Expression vectors for full-length human huntingtin were synthesized at GenScript. Full-length wildtype (wt) HTT contains 23 consecutive CAG repeats. Mutant huntingtin (mHTT) with 90 polyglutamines, was cloned by inserting further CAG repeats into the wildtype cDNA. … Get A Quote

摘要

Mammalian transglutaminases (TGs) catalyze calcium-dependent irreversible posttranslational modifications of proteins and their enzymatic activities contribute to the pathogenesis of several human neurodegenerative diseases. Although different transglutaminases are found in many different tissues, the TG6 isoform is mostly expressed in the CNS. The present study was embarked on/undertaken to investigate expression, distribution and activity of transglutaminases in Huntington disease transgenic rodent models, with a focus on analyzing the involvement of TG6 in the age- and genotype-specific pathological features relating to disease progression in HD transgenic mice and a tgHD transgenic rat model using biochemic... More

关键词

Huntington disease, neurodegeneration, rodent transgenic animal models, transglutaminase isoform 6