Nucleocytoplasmic transport defects in neurodegeneration – cause or consequence?

Nucleocytoplasmic transport defects in neurodegeneration – cause or consequence?

Publication date: May 29, 2019

Defects in nucleocytoplasmic transport have been associated with several neurodegenerative disorders and, in particular, the formation of pathological protein aggregates characteristic for the respective disease. However, whether impaired nucleocytoplasmic transport is a consequence of such aggregates or rather contributes to their formation is still mostly unclear. In this review, we summarize recent findings how both soluble and stationary components of the nucleocytoplasmic transport machinery are altered in neurodegenerative diseases, in particular amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), Alzheimer’s disease (AD) and Huntington’s disease (HD). We discuss the functional significance of the observed defects for nucleocytoplasmic transport of proteins and mRNAs. Moreover, we highlight interesting parallels observed in physiological ageing and the premature ageing syndrome progeria and propose that they that might provide mechanistic insights also for neurodegenerative processes.

Hutten, S. and Dormann, D. Nucleocytoplasmic transport defects in neurodegeneration – cause or consequence? 06511. 2019 Semin Cell Dev Biol.

Concepts Keywords
ALS Senescence
Alzheimer Branches of biology
Amyotrophic Lateral Sclerosis Degenerative disease
Frontotemporal Dementia Neurodegeneration
Huntington Progeria
MRNAs Frontotemporal dementia
Neurodegeneration Nucleoporin 214
Neurodegenerative Diseases
Neurodegenerative Disorders


Type Source Name
pathway BSID Nuclear pore complex
disease DOID progeria
disease MESH progeria
disease DOID syndrome
disease MESH syndrome
disease DOID frontotemporal dementia
disease MESH frontotemporal dementia
disease DOID amyotrophic lateral sclerosis
disease MESH amyotrophic lateral sclerosis
pathway BSID Neurodegenerative Diseases
disease MESH neurodegenerative disorders
disease MESH defects


Original Article

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