Logo image
DCTN1 mutations in Perry syndrome
Journal article   Open access   Peer reviewed

DCTN1 mutations in Perry syndrome

Matthew J Farrer, Mary M Hulihan, Jennifer M Kachergus, Justus Dächsel, A. Jon Stoessl, Linda L Grantier, Susan Calne, Donald B Calne, Bernard Lechevalier, Francoise Chapon, …
Nature genetics, Vol.41(2), pp.163-165
02/2009
DOI: 10.1038/ng.293
PMCID: PMC2813485
PMID: 19136952
url
https://doi.org/10.1038/ng.293View
Published (Version of record) Open Access

Abstract

Perry syndrome consists of early-onset parkinsonism, depression, severe weight loss and hypoventilation, in which brain pathology is characterized by TDP-43 immunostaining. Through genome-wide linkage analysis we have identified five disease-segregating dynactin ( DCTN1 ) CAP-Gly domain substitutions in 8 families that diminish microtubule binding and lead to intracytoplasmic inclusions. DCTN1 mutations were previously associated with motor neuron disease but can underlie the selective vulnerability of other neuronal populations in distinct neurodegenerative disorders.
Perry syndrome neurodegeneration Dynactin parkinsonism DCTN1 TDP-43

Details

Metrics

Logo image