Drosophila as an In Vivo Model for Human Neurodegenerative Disease

L McGurk, A Berson, NM Bonini - Genetics, 2015 - academic.oup.com
With the increase in the ageing population, neurodegenerative disease is devastating to
families and poses a huge burden on society. The brain and spinal cord are extraordinarily …

ER stress and diseases

H Yoshida - The FEBS journal, 2007 - Wiley Online Library
Proteins synthesized in the endoplasmic reticulum (ER) are properly folded with the
assistance of ER chaperones. Malfolded proteins are disposed of by ER‐associated protein …

Therapeutic potential of mood stabilizers lithium and valproic acid: beyond bipolar disorder

CT Chiu, Z Wang, JG Hunsberger, DM Chuang… - Pharmacological …, 2013 - Elsevier
The mood stabilizers lithium and valproic acid (VPA) are traditionally used to treat bipolar
disorder (BD), a severe mental illness arising from complex interactions between genes and …

Protein aggregates in Huntington's disease

M Arrasate, S Finkbeiner - Experimental neurology, 2012 - Elsevier
Huntington's disease (HD) is an incurable neurodegenerative disease characterized by
abnormal motor movements, personality changes, and early death. HD is caused by a …

Modulation of neurodegeneration by molecular chaperones

PJ Muchowski, JL Wacker - Nature Reviews Neuroscience, 2005 - nature.com
Many neurodegenerative disorders are characterized by conformational changes in proteins
that result in misfolding, aggregation and intra-or extra-neuronal accumulation of amyloid …

The neuroprotective properties of calorie restriction, the ketogenic diet, and ketone bodies

M Maalouf, JM Rho, MP Mattson - Brain research reviews, 2009 - Elsevier
Both calorie restriction and the ketogenic diet possess broad therapeutic potential in various
clinical settings and in various animal models of neurological disease. Following calorie …

Comparative genomics and molecular dynamics of DNA repeats in eukaryotes

GF Richard, A Kerrest, B Dujon - Microbiology and molecular …, 2008 - Am Soc Microbiol
Repeated elements can be widely abundant in eukaryotic genomes, composing more than
50% of the human genome, for example. It is possible to classify repeated sequences into …

Diseases of unstable repeat expansion: mechanisms and common principles

JR Gatchel, HY Zoghbi - Nature Reviews Genetics, 2005 - nature.com
The list of developmental and degenerative diseases that are caused by expansion of
unstable repeats continues to grow, and is now approaching 20 disorders. The pathogenic …

[HTML][HTML] Pathogenesis of SCA3 and implications for other polyglutamine diseases

HS McLoughlin, LR Moore, HL Paulson - Neurobiology of disease, 2020 - Elsevier
Tandem repeat diseases include the neurodegenerative disorders known as polyglutamine
(polyQ) diseases, caused by CAG repeat expansions in the coding regions of the respective …

A DNAJB chaperone subfamily with HDAC-dependent activities suppresses toxic protein aggregation

J Hageman, MA Rujano, MAWH Van Waarde, V Kakkar… - Molecular cell, 2010 - cell.com
Misfolding and aggregation are associated with cytotoxicity in several protein folding
diseases. A large network of molecular chaperones ensures protein quality control. Here, we …