M George, M Tharakan, J Culberson, AP Reddy… - Ageing Research …, 2022 - Elsevier
Abstract Nuclear Factor-Erythroid Factor 2 (Nrf2) is an important transcription factor that regulates the expression of large number of genes in healthy and disease states. Nrf2 is …
Huntingtin (HTT) is now a famous protein because an abnormal expansion of a glutamine stretch (polyQ) in its N-terminal sequence leads to the devastating neurodegenerative …
The ability to catalytically cleave protein substrates after synthesis is fundamental for all forms of life. Accordingly, site-specific proteolysis is one of the most important post …
R Tenchov, JM Sasso, QA Zhou - ACS Chemical Neuroscience, 2024 - ACS Publications
Polyglutamine (polyQ) diseases are a group of inherited neurodegenerative disorders caused by expanded cytosine-adenine-guanine (CAG) repeats encoding proteins with …
Mitochondrial dysfunction has been described as an early pathological mechanism delineating the selective neurodegeneration that occurs in Huntington's disease (HD), a …
LM Thompson, CT Aiken, LS Kaltenbach… - Journal of Cell …, 2009 - rupress.org
Expansion of the polyglutamine repeat within the protein Huntingtin (Htt) causes Huntington's disease, a neurodegenerative disease associated with aging and the …
PG Jobin, GS Butler, CM Overall - … et Biophysica Acta (BBA)-Molecular Cell …, 2017 - Elsevier
Adaption of a single protein to perform multiple independent functions facilitates functional plasticity of the proteome allowing a limited number of protein-coding genes to perform a …
Investigations of synaptic transmission and plasticity in mouse models of Huntington's disease (HD) demonstrate neuronal dysfunction long before the onset of classical disease …
C Landles, K Sathasivam, A Weiss, B Woodman… - Journal of Biological …, 2010 - ASBMB
Huntingtin proteolysis has been implicated in the molecular pathogenesis of Huntington disease (HD). Despite an intense effort, the identity of the pathogenic smallest N-terminal …