Mitochondrial dysfunction and biogenesis in neurodegenerative diseases: pathogenesis and treatment

M Golpich, E Amini, Z Mohamed… - CNS neuroscience & …, 2017 - Wiley Online Library
Neurodegenerative diseases are a heterogeneous group of disorders that are incurable and
characterized by the progressive degeneration of the function and structure of the central …

Huntington disease

GP Bates, R Dorsey, JF Gusella, MR Hayden… - Nature reviews Disease …, 2015 - nature.com
Huntington disease is devastating to patients and their families—with autosomal dominant
inheritance, onset typically in the prime of adult life, progressive course, and a combination …

Integrated genomics and proteomics define huntingtin CAG length–dependent networks in mice

P Langfelder, JP Cantle, D Chatzopoulou… - Nature …, 2016 - nature.com
To gain insight into how mutant huntingtin (mHtt) CAG repeat length modifies Huntington's
disease (HD) pathogenesis, we profiled mRNA in over 600 brain and peripheral tissue …

Mitochondrial diseases of the brain

RK Chaturvedi, MF Beal - Free Radical Biology and Medicine, 2013 - Elsevier
Neurodegenerative disorders are debilitating diseases of the brain, characterized by
behavioral, motor and cognitive impairments. Ample evidence underpins mitochondrial …

Huntington disease: natural history, biomarkers and prospects for therapeutics

CA Ross, EH Aylward, EJ Wild, DR Langbehn… - Nature Reviews …, 2014 - nature.com
Huntington disease (HD) can be seen as a model neurodegenerative disorder, in that it is
caused by a single genetic mutation and is amenable to predictive genetic testing, with …

Trinucleotide repeat disorders

HT Orr, HY Zoghbi - Annu. Rev. Neurosci., 2007 - annualreviews.org
The discovery that expansion of unstable repeats can cause a variety of neurological
disorders has changed the landscape of disease-oriented research for several forms of …

PGC-1α rescues Huntington's disease proteotoxicity by preventing oxidative stress and promoting TFEB function

T Tsunemi, TD Ashe, BE Morrison… - Science translational …, 2012 - science.org
Huntington's disease (HD) is caused by CAG repeat expansions in the (huntingtin htt) gene,
yielding proteins containing polyglutamine repeats that become misfolded and resist …

Ageing and neuronal vulnerability

MP Mattson, T Magnus - Nature reviews neuroscience, 2006 - nature.com
Everyone ages, but only some will develop a neurodegenerative disorder in the process.
Disease might occur when cells fail to respond adaptively to age-related increases in …

[HTML][HTML] Induced pluripotent stem cells from patients with Huntington's disease show CAG-repeat-expansion-associated phenotypes

Hd iPsc Consortium - Cell stem cell, 2012 - cell.com
Huntington's disease (HD) is an inherited neurodegenerative disorder caused by an
expanded stretch of CAG trinucleotide repeats that results in neuronal dysfunction and …

The molecular and cellular basis of copper dysregulation and its relationship with human pathologies

MT Maung, A Carlson, M Olea‐Flores… - The FASEB …, 2021 - Wiley Online Library
Copper (Cu) is an essential micronutrient required for the activity of redox‐active enzymes
involved in critical metabolic reactions, signaling pathways, and biological functions …