Molecular mechanisms and potential therapeutical targets in Huntington's disease

C Zuccato, M Valenza, E Cattaneo - Physiological reviews, 2010 - journals.physiology.org
Huntington's disease (HD) is a neurodegenerative disorder caused by a CAG repeat
expansion in the gene encoding for huntingtin protein. A lot has been learned about this …

Metabolic dysfunction in Alzheimer's disease and related neurodegenerative disorders

H Cai, W Cong, S Ji, S Rothman… - Current Alzheimer …, 2012 - ingentaconnect.com
Alzheimer's disease and other related neurodegenerative diseases are highly debilitating
disorders that affect millions of people worldwide. Efforts towards develo** effective …

Large-scale association analyses identify new loci influencing glycemic traits and provide insight into the underlying biological pathways

RA Scott, V Lagou, RP Welch, E Wheeler… - Nature …, 2012 - nature.com
Through genome-wide association meta-analyses of up to 133,010 individuals of European
ancestry without diabetes, including individuals newly genotyped using the Metabochip, we …

Beyond the brain: widespread pathology in Huntington's disease

JMM van der Burg, M Björkqvist, P Brundin - The Lancet Neurology, 2009 - thelancet.com
Huntington's disease (HD) is an inherited neurodegenerative disorder caused by a
polyglutamine stretch in the huntingtin protein. Today, more than 15 years after the genetic …

Insulin, IGF-1 and GLP-1 signaling in neurodegenerative disorders: targets for disease modification?

F Bassil, PO Fernagut, E Bezard, WG Meissner - Progress in neurobiology, 2014 - Elsevier
Abstract Insulin and Insulin Growth Factor-1 (IGF-1) play a major role in body homeostasis
and glucose regulation. They also have paracrine/autocrine functions in the brain. The …

Significance of brain glucose hypometabolism, altered insulin signal transduction, and insulin resistance in several neurological diseases

E Blázquez, V Hurtado-Carneiro… - Frontiers in …, 2022 - frontiersin.org
Several neurological diseases share pathological alterations, even though they differ in their
etiology. Neuroinflammation, altered brain glucose metabolism, oxidative stress …

The molecular genetics of Huntington disease—a history

GP Bates - Nature Reviews Genetics, 2005 - nature.com
The Huntington disease gene was mapped to human chromosome 4p in 1983 and 10 years
later the pathogenic mutation was identified as a CAG-repeat expansion. Our current …

The use of the R6 transgenic mouse models of Huntington's disease in attempts to develop novel therapeutic strategies

JY Li, N Popovic, P Brundin - NeuroRx, 2005 - Elsevier
Huntington's disease (HD) is a genetic neurodegenerative disorder. Since identification of
the disease-causing gene in 1993, a number of genetically modified animal models of HD …

Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence

U Bichelmeier, T Schmidt, J Hübener, J Boy… - Journal of …, 2007 - Soc Neuroscience
Spinocerebellar ataxia type 3 (SCA3) is an autosomal dominantly inherited
neurodegenerative disorder caused by the expansion of a CAG repeat in the MJD1 gene …

Mutant huntingtin impairs immune cell migration in Huntington disease

W Kwan, U Träger, D Davalos, A Chou… - The Journal of …, 2012 - Am Soc Clin Investig
In Huntington disease (HD), immune cells are activated before symptoms arise; however, it
is unclear how the expression of mutant huntingtin (htt) compromises the normal functions of …