The biology of proteostasis in aging and disease

J Labbadia, RI Morimoto - Annual review of biochemistry, 2015 - annualreviews.org
Loss of protein homeostasis (proteostasis) is a common feature of aging and disease that is
characterized by the appearance of nonnative protein aggregates in various tissues. Protein …

Huntington's disease: underlying molecular mechanisms and emerging concepts

J Labbadia, RI Morimoto - Trends in biochemical sciences, 2013 - cell.com
Huntington's disease (HD) is a progressive neurodegenerative disorder for which no
disease modifying treatments exist. Many molecular changes and cellular consequences …

[HTML][HTML] Repression of the heat shock response is a programmed event at the onset of reproduction

J Labbadia, RI Morimoto - Molecular cell, 2015 - cell.com
The heat shock response (HSR) is essential for proteostasis and cellular health. In
metazoans, aging is associated with a decline in quality control, thus increasing the risk for …

NF-Y and the transcriptional activation of CCAAT promoters

D Dolfini, R Gatta, R Mantovani - Critical reviews in biochemistry …, 2012 - Taylor & Francis
The CCAAT box promoter element and NF-Y, the transcription factor (TF) that binds to it,
were among the first cis-elements and trans-acting factors identified; their interplay is …

The CAG–polyglutamine repeat diseases: a clinical, molecular, genetic, and pathophysiologic nosology

CA Stoyas, AR La Spada - Handbook of clinical neurology, 2018 - Elsevier
Throughout the genome, unstable tandem nucleotide repeats can expand to cause a variety
of neurologic disorders. Expansion of a CAG triplet repeat within a coding exon gives rise to …

Sequestration of cellular interacting partners by protein aggregates: implication in a loss‐of‐function pathology

H Yang, HY Hu - The FEBS journal, 2016 - Wiley Online Library
Protein misfolding and aggregation are a hallmark of several neurodegenerative diseases
(ND s). However, how protein aggregation leads to cytotoxicity and neurodegeneration is …

Age-dependent decrease in chaperone activity impairs MANF expression, leading to Purkinje cell degeneration in inducible SCA17 mice

S Yang, S Huang, MA Gaertig, XJ Li, S Li - Neuron, 2014 - cell.com
Although protein-misfolding-mediated neurodegenerative diseases have been linked to
aging, how aging contributes to selective neurodegeneration remains unclear. We …

Ataxin-2 Regulates RGS8 Translation in a New BAC-SCA2 Transgenic Mouse Model

W Dansithong, S Paul, KP Figueroa, MD Rinehart… - PLoS …, 2015 - journals.plos.org
Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant disorder with progressive
degeneration of cerebellar Purkinje cells (PCs) and other neurons caused by expansion of a …

[HTML][HTML] Chronic treatment with 17-DMAG improves balance and coordination in a new mouse model of Machado-Joseph disease

A Silva-Fernandes, S Duarte-Silva, A Neves-Carvalho… - …, 2014 - Elsevier
Machado-Joseph disease (MJD) or spinocerebellar ataxia type 3 (SCA3) is a
neurodegenerative disease currently with no treatment. We describe a novel mouse model …

The role (s) of NF-Y in development and differentiation

D Dolfini, C Imbriano, R Mantovani - Cell Death & Differentiation, 2024 - nature.com
NF-Y is a conserved sequence-specific trimeric Transcription Factor-TF-binding to the
CCAAT element. We review here the role (s) in development, from pre-implantation embryo …