Adult neurogenesis in neurodegenerative diseases

B Winner, J Winkler - Cold Spring Harbor perspectives in …, 2015 - cshperspectives.cshlp.org
Adult neurogenesis is limited to specific brain regions in the mammalian brain, such as the
hippocampal dentate gyrus and the subventricular zone/olfactory bulb system. Alterations in …

α‐Synuclein oligomers and clinical implications for Parkinson disease

LV Kalia, SK Kalia, PJ McLean, AM Lozano… - Annals of …, 2013 - Wiley Online Library
Protein aggregation within the central nervous system has been recognized as a defining
feature of neurodegenerative diseases since the early 20th century. Since that time, there …

The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington's disease patients

A Neueder, C Landles, R Ghosh, D Howland… - Scientific reports, 2017 - nature.com
We have previously shown that exon 1 of the huntingtin gene does not always splice to exon
2 resulting in the production of a small polyadenylated mRNA (HTTexon1) that encodes the …

Neurodegenerative disease and adult neurogenesis

B Winner, Z Kohl, FH Gage - European Journal of Neuroscience, 2011 - Wiley Online Library
The generation and cell death of newly generated cells have critical roles in brain
development and maintenance in the embryonic and adult brain. Alterations in these …

Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage

U Shirendeb, AP Reddy, M Manczak… - Human molecular …, 2011 - academic.oup.com
The purpose of our study was to determine the relationship between mutant huntingtin (Htt)
and mitochondrial dynamics in the progression of Huntington's disease (HD). We measured …

The structure of pathogenic huntingtin exon 1 defines the bases of its aggregation propensity

CA Elena-Real, A Sagar, A Urbanek… - Nature structural & …, 2023 - nature.com
Huntington's disease is a neurodegenerative disorder caused by a CAG expansion in the
first exon of the HTT gene, resulting in an extended polyglutamine (poly-Q) tract in huntingtin …

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 …

Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease

C Landles, K Sathasivam, A Weiss, B Woodman… - Journal of Biological …, 2010 - ASBMB
Huntingtin proteolysis has been implicated in the molecular pathogenesis of Huntington
disease (HD). Despite an intense effort, the identity of the pathogenic smallest N-terminal …

Soluble mutant huntingtin drives early human pathogenesis in Huntington's disease

A Miguez, C Gomis, C Vila… - Cellular and Molecular …, 2023 - Springer
Huntington's disease (HD) is an incurable inherited brain disorder characterised by massive
degeneration of striatal neurons, which correlates with abnormal accumulation of misfolded …

Proteins containing expanded polyglutamine tracts and neurodegenerative disease

A Adegbuyiro, F Sedighi, AW Pilkington IV… - Biochemistry, 2017 - ACS Publications
Several hereditary neurological and neuromuscular diseases are caused by an abnormal
expansion of trinucleotide repeats. To date, there have been 10 of these trinucleotide repeat …