Calcium pumps in health and disease

M Brini, E Carafoli - Physiological reviews, 2009 - journals.physiology.org
Ca2+-ATPases (pumps) are key actors in the regulation of Ca2+ in eukaryotic cells and are
thus essential to the correct functioning of the cell machinery. They have high affinity for …

Calcium signaling and neurodegenerative diseases

I Bezprozvanny - Trends in molecular medicine, 2009 - cell.com
Neurodegenerative disorders, such as Alzheimer's disease (AD), Parkinson's disease (PD),
amyotrophic lateral sclerosis (ALS), Huntington's disease (HD) and spinocerebellar ataxias …

Nonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in Huntington's disease mice

RL Boudreau, JL McBride, I Martins, S Shen, Y Xing… - Molecular Therapy, 2009 - cell.com
Huntington's disease (HD) is a fatal neurodegenerative disease caused by mutant huntingtin
(htt) protein, and there are currently no effective treatments. Recently, we and others …

Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models

L Menalled, BF El-Khodor, M Patry… - Neurobiology of …, 2009 - Elsevier
Huntington's disease (HD) is one of the few neurodegenerative diseases with a known
genetic cause, knowledge that has enabled the creation of animal models using genetic …

Distinct neuroinflammatory profile in post-mortem human Huntington's disease

A Silvestroni, RLM Faull, AD Strand, T Möller - Neuroreport, 2009 - journals.lww.com
Neuroinflammation is a prominent feature of many neurodegenerative diseases, however,
little is known about neuroinflammation in Huntington's disease. We used quantitative real …

The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies

PO Bauer, N Nukina - Journal of neurochemistry, 2009 - Wiley Online Library
Expansion of CAG trinucleotide repeat within the coding region of several genes results in
the production of proteins with expanded polyglutamine (PolyQ) stretch. The expression of …

Formation of Polyglutamine Inclusions in a Wide Range of Non-CNS Tissues in the HdhQ150 Knock-In Mouse Model of Huntington's Disease

H Moffitt, GD McPhail, B Woodman, C Hobbs… - PloS one, 2009 - journals.plos.org
Background Huntington's disease (HD) is an inherited progressive neurodegenerative
disorder caused by a CAG repeat expansion in the ubiquitously expressed HD gene …

Gene dysregulation in Huntington's disease: REST, microRNAs and beyond

R Johnson, NJ Buckley - Neuromolecular medicine, 2009 - Springer
Huntington's disease (HD) is an incurable, fatal neurodegenerative disorder that is caused
by a polyglutamine expansion in the huntingtin (Htt) protein. Neuronal death in the striatum …

DNA breakage and induction of DNA damage response proteins precede the appearance of visible mutant huntingtin aggregates

J Illuzzi, S Yerkes, H Parekh‐Olmedo… - Journal of …, 2009 - Wiley Online Library
Huntington's disease (HD) is a neurodegenerative disorder that follows an autosomal‐
dominant inheritance pattern. The pathogenesis of the disease depends on the degree of …

A large number of protein expression changes occur early in life and precede phenotype onset in a mouse model for Huntington disease* S

C Zabel, L Mao, B Woodman, M Rohe… - Molecular & Cellular …, 2009 - ASBMB
Huntington disease (HD) is fatal in humans within 15–20 years of symptomatic disease.
Although late stage HD has been studied extensively, protein expression changes that occur …