Pathogenic huntingtin inhibits fast axonal transport by activating JNK3 and phosphorylating kinesin

GA Morfini, YM You, SL Pollema, A Kaminska… - Nature …, 2009 - nature.com
Selected vulnerability of neurons in Huntington's disease suggests that alterations occur in a
cellular process that is particularly critical for neuronal function. Supporting this idea …

Mouse models of Huntington's disease

LB Menalled, MF Chesselet - Trends in pharmacological sciences, 2002 - cell.com
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder. In 1993
the mutation that causes HD was identified as an unstable expansion of CAG repeats in the …

Interaction of Huntington disease protein with transcriptional activator Sp1

SH Li, AL Cheng, H Zhou, S Lam, M Rao… - Molecular and cellular …, 2002 - Taylor & Francis
Polyglutamine expansion causes Huntington disease (HD) and at least seven other
neurodegenerative diseases. In HD, N-terminal fragments of huntingtin with an expanded …

Lessons from animal models of Huntington's disease

DC Rubinsztein - TRENDS in Genetics, 2002 - cell.com
Huntington's disease (HD) is an autosomal–dominant neurodegenerative disorder caused
by a CAG trinucleotide repeat expansion in the HD gene. The expanded repeats are …

Genetic modifiers of Huntington's disease

JF Gusella, ME MacDonald, JM Lee - Movement Disorders, 2014 - Wiley Online Library
Huntington's disease (HD) is a devastating neurodegenerative disorder that directly affects
more than 1 in 10,000 persons in Western societies but, as a family disorder with a long …

Huntington's disease: can mice lead the way to treatment?

ZR Crook, D Housman - Neuron, 2011 - cell.com
Mouse models for Huntington's Disease (HD) and HD patients demonstrate motor and
behavioral dysfunctions, such as progressive loss of coordination and memory, and share …

ABHD12 controls brain lysophosphatidylserine pathways that are deregulated in a murine model of the neurodegenerative disease PHARC

JL Blankman, JZ Long, SA Trauger… - Proceedings of the …, 2013 - National Acad Sciences
Advances in human genetics are leading to the discovery of new disease-causing mutations
at a remarkable rate. Many such mutations, however, occur in genes that encode for proteins …

[HTML][HTML] Antioxidants in Huntington's disease

A Johri, MF Beal - Biochimica et Biophysica Acta (BBA)-Molecular Basis …, 2012 - Elsevier
Huntington's disease (HD) is a prototypical neurodegenerative disease in which there is
selective neuronal degeneration, which leads to progressive disability, manifesting itself as …

Intergenerational and striatal CAG repeat instability in Huntington's disease knock-in mice involve different DNA repair genes

E Dragileva, A Hendricks, A Teed, T Gillis… - Neurobiology of …, 2009 - Elsevier
Modifying the length of the Huntington's disease (HD) CAG repeat, the major determinant of
age of disease onset, is an attractive therapeutic approach. To explore this we are …

Juvenile onset Huntington's disease—clinical and research perspectives

MA Nance, RH Myers - Mental retardation and developmental …, 2001 - Wiley Online Library
Huntington's disease (HD) is an inherited neurodegenerative disorder. The mutation which
causes the disease is an expansion in the number of repetitions of three nucleotides, C, A …