Expression of normal and mutant huntingtin in the developing brain

PG Bhide, M Day, E Sapp, C Schwarz… - Journal of …, 1996 - Soc Neuroscience
Huntington's disease (HD) is caused by a genetic mutation that results in a polyglutamine
expansion in huntingtin. The time course of neuronal loss in the HD striatum and other …

Expression of mutant huntingtin in mouse brain astrocytes causes age-dependent neurological symptoms

J Bradford, JY Shin, M Roberts… - Proceedings of the …, 2009 - National Acad Sciences
Huntington disease (HD) is an inherited neurological disorder caused by a polyglutamine
expansion in the protein huntingtin and is characterized by selective neurodegeneration that …

Dysfunction of wild-type huntingtin in Huntington disease

E Cattaneo - Physiology, 2003 - journals.physiology.org
Huntingtin is the protein involved in Huntington disease (HD), an inherited
neurodegenerative disease. Research activities have focused on the abnormal functions of …

[HTML][HTML] The biological function of the Huntingtin protein and its relevance to Huntington's Disease pathology

J Schulte, JT Littleton - Current trends in neurology, 2011 - ncbi.nlm.nih.gov
Huntington's Disease is an adult-onset dominant heritable disorder characterized by
progressive psychiatric disruption, cognitive deficits, and loss of motor coordination. It is …

Huntington disease: new insights on the role of huntingtin cleavage

CL Wellington, BR Leavitt, MR Hayden - Advances in Research on …, 2000 - Springer
Huntington Disease (HD) results from polyglutamine expansion within the N-terminus of
huntingtin. We have produced yeast artificial chromosome (YAC) transgenic mice …

Differential expression of normal and mutant Huntington's disease gene alleles

F Persichetti, L Carlee, PW Faber, SM McNeil… - Neurobiology of …, 1996 - Elsevier
Huntingtin expression was examined by Western blot and immunoprecipitation studies of
lymphoblastoid cell lines from Huntington's disease (HD) homozygotes, heterozygotes, and …

Depletion of wild‐type huntingtin in mouse models of neurologic diseases

Y Zhang, M Li, M Drozda, M Chen, S Ren… - Journal of …, 2003 - Wiley Online Library
Huntington's disease (HD) is caused by a mutation in the gene encoding for huntingtin
resulting in selective neuronal degeneration. Because HD is an autosomal dominant …

Huntingtin localization in brains of normal and Huntington's disease patients

E Sapp, C Schwarz, K Chase, PG Bhide… - Annals of Neurology …, 1997 - Wiley Online Library
The immunohistochemical localization of huntingtin was examined in the Huntington's
disease (HD) brain with an antibody that recognizes the wild‐type and mutant proteins …

Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity

H Li, SH Li, H Johnston, PF Shelbourne, XJ Li - Nature genetics, 2000 - nature.com
Huntington disease (HD) is caused by expansion of a glutamine repeat in the amino-
terminal region of huntingtin. Despite its widespread expression, mutant huntingtin induces …

Calcium-dependent cleavage of endogenous wild-type huntingtin in primary cortical neurons

D Goffredo, D Rigamonti, M Tartari, A De Micheli… - Journal of Biological …, 2002 - ASBMB
Huntington's disease (HD) is caused by a polyglutamine expansion in the amino-terminal
region of huntingtin. Mutant huntingtin is proteolytically cleaved by caspases, generating …