Rodent genetic models of Huntington disease
Huntington disease (HD) is a dominantly inherited human neurodegenerative disorder
characterized by motor deficits, cognitive impairment, and psychiatric symptoms leading to …
characterized by motor deficits, cognitive impairment, and psychiatric symptoms leading to …
Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice
M Gray, DI Shirasaki, C Cepeda, VM André… - Journal of …, 2008 - Soc Neuroscience
To elucidate the pathogenic mechanisms in Huntington's disease (HD) elicited by
expression of full-length human mutant huntingtin (fl-mhtt), a bacterial artificial chromosome …
expression of full-length human mutant huntingtin (fl-mhtt), a bacterial artificial chromosome …
[HTML][HTML] Suppression of neurodegeneration and increased neurotransmission caused by expanded full-length huntingtin accumulating in the cytoplasm
E Romero, GH Cha, P Verstreken, CV Ly, RE Hughes… - Neuron, 2008 - cell.com
Huntington's disease (HD) is a dominantly inherited neurodegenerative disorder caused by
expansion of a translated CAG repeat in the N terminus of the huntingtin (htt) protein. Here …
expansion of a translated CAG repeat in the N terminus of the huntingtin (htt) protein. Here …
A critical evaluation of adenosine A2A receptors as potentially “druggable” targets in Huntington's disease
P Popoli, D Blum, MR Domenici… - Current …, 2008 - ingentaconnect.com
Huntington's disease (HD) is a dominantly inherited neurodegenerative disorder caused by
the expansion of a polymorphic CAG trinucleotide repeat encoding a poly-glutamine tract …
the expansion of a polymorphic CAG trinucleotide repeat encoding a poly-glutamine tract …
Automated deformation analysis in the YAC128 Huntington disease mouse model
The YAC128 mouse recapitulates many of the clinical features of Huntington disease (HD),
including selective neuropathology with neuronal loss. Here we investigate whether …
including selective neuropathology with neuronal loss. Here we investigate whether …
Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease
CE Wang, S Tydlacka, AL Orr, SH Yang… - Human molecular …, 2008 - academic.oup.com
A number of mouse models expressing mutant huntingtin (htt) with an expanded
polyglutamine (polyQ) domain are useful for studying the pathogenesis of Huntington's …
polyglutamine (polyQ) domain are useful for studying the pathogenesis of Huntington's …
Activated caspase-6 and caspase-6-cleaved fragments of huntingtin specifically colocalize in the nucleus
Proteolysis of mutant huntingtin is crucial to the development of Huntington disease (HD).
Specifically preventing proteolysis at the capase-6 (C6) consensus sequence at amino acid …
Specifically preventing proteolysis at the capase-6 (C6) consensus sequence at amino acid …
Cortical thickness measured from MRI in the YAC128 mouse model of Huntington's disease
A recent study found differences in localised regions of the cortex between the YAC128
mouse model of Huntington's Disease (HD) and wild-type mice. There are, however, few …
mouse model of Huntington's Disease (HD) and wild-type mice. There are, however, few …
Compensatory changes in the ubiquitin–proteasome system, brain-derived neurotrophic factor and mitochondrial complex II/III in YAC72 and R6/2 transgenic mice …
Intraneuronal protein aggregates of the mutated huntingtin in Huntington's disease (HD)
brains suggest an overload and/or dysfunction of the ubiquitin–proteasome system (UPS) …
brains suggest an overload and/or dysfunction of the ubiquitin–proteasome system (UPS) …
Striosome-matrix pathology and motor deficits in the YAC128 mouse model of Huntington's disease
C Lawhorn, DM Smith, LL Brown - Neurobiology of disease, 2008 - Elsevier
Huntington's disease is characterized by striatal degeneration and progressive motor
deficits. To examine striatal compartment-specific pathology and its relation to motor …
deficits. To examine striatal compartment-specific pathology and its relation to motor …