Selective vulnerability in neurodegenerative diseases

H Fu, J Hardy, KE Duff - Nature neuroscience, 2018 - nature.com
Neurodegenerative diseases have two general characteristics that are so fundamental we
usually take them for granted. The first is that the pathology associated with the disease only …

Neurodegenerative diseases–is metabolic deficiency the root cause?

VR Muddapu, SAP Dharshini… - Frontiers in …, 2020 - frontiersin.org
Neurodegenerative diseases, including Alzheimer, Parkinson, Huntington, and amyotrophic
lateral sclerosis, are a prominent class of neurological diseases currently without a cure …

Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

M Arrasate, S Mitra, ES Schweitzer, MR Segal… - nature, 2004 - nature.com
Huntington's disease is caused by an abnormal polyglutamine expansion within the protein
huntingtin and is characterized by microscopic inclusion bodies of aggregated huntingtin …

Pathophysiology of Huntington's disease: time-dependent alterations in synaptic and receptor function

LA Raymond, VM André, C Cepeda, CM Gladding… - Neuroscience, 2011 - Elsevier
Huntington's disease (HD) is a progressive, fatal neurological condition caused by an
expansion of CAG (glutamine) repeats in the coding region of the Huntington gene. To date …

Genetics and neuropathology of Huntington's disease

A Reiner, I Dragatsis, P Dietrich - International review of neurobiology, 2011 - Elsevier
Huntington's disease (HD) is an autosomal dominant progressive neurodegenerative
disorder that prominently affects the basal ganglia, leading to affective, cognitive, behavioral …

Huntington's disease

S Finkbeiner - Cold Spring Harbor perspectives in …, 2011 - cshperspectives.cshlp.org
Huntington's disease (HD) is the most common inherited neurodegenerative disease and is
characterized by uncontrolled excessive motor movements and cognitive and emotional …

Huntingtin aggregate-associated axonal degeneration is an early pathological event in Huntington's disease mice

H Li, SH Li, ZX Yu, P Shelbourne… - Journal of Neuroscience, 2001 - Soc Neuroscience
Huntington's disease (HD) is characterized by the selective loss of striatal projection
neurons. In early stages of HD, neurodegeneration preferentially occurs in the lateral globus …

Brain-derived neurotrophic factor regulates the onset and severity of motor dysfunction associated with enkephalinergic neuronal degeneration in Huntington's …

JM Canals, JR Pineda, JF Torres-Peraza… - Journal of …, 2004 - Soc Neuroscience
The mechanism that controls the selective vulnerability of striatal neurons in Huntington's
disease is unclear. Brain-derived neurotrophic factor (BDNF) protects striatal neurons and is …

The corticostriatal pathway in Huntington's disease

C Cepeda, N Wu, VM André, DM Cummings… - Progress in …, 2007 - Elsevier
The corticostriatal pathway provides most of the excitatory glutamatergic input into the
striatum and it plays an important role in the development of the phenotype of Huntington's …

Replicating Huntington's disease phenotype in experimental animals

E Brouillet, F Conde, MF Beal, P Hantraye - Progress in neurobiology, 1999 - Elsevier
Huntington's disease (HD) is an inherited, autosomal dominant, neurodegenerative disorder
characterized by involuntary choreiform movements, cognitive decline and a progressive …