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Journal ArticleDOI

Axonal transport deficits and neurodegenerative diseases

TLDR
The current state of knowledge about axonal transport defects that might contribute to the pathogenesis of particular neurodegenerative diseases are reviewed.
Abstract
The intracellular transport of organelles along an axon is crucial for the maintenance and function of a neuron. Anterograde axonal transport has a role in supplying proteins and lipids to the distal synapse and mitochondria for local energy requirements, whereas retrograde transport is involved in the clearance of misfolded and aggregated proteins from the axon and the intracellular transport of distal trophic signals to the soma. Axonal transport can be affected by alterations to various components of the transport machinery. Here, we review the current state of knowledge about axonal transport defects that might contribute to the pathogenesis of particular neurodegenerative diseases.

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Citations
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Journal ArticleDOI

Selective neuronal vulnerability in Parkinson disease.

TL;DR: The evidence for and against the spreading LP model are discussed, as well as evidence that cell-autonomous factors govern both α-syn pathology and neuronal death.
Journal ArticleDOI

Roles of tau protein in health and disease.

TL;DR: It is important to fully understand the range of neuronal functions attributed to tau, since this will provide vital information on its involvement in the development and pathogenesis of disease, and enable determination of which critical molecular pathways should be targeted by potential therapeutic agents developed for the treatment of tauopathies.
Journal ArticleDOI

Axonal transport: cargo-specific mechanisms of motility and regulation

TL;DR: An overview of axonal transport pathways is provided and their role in neuronal function is discussed and Retrograde transport, which plays a major role in neurotrophic and injury response signaling, is discussed.
Journal ArticleDOI

Building the Neuronal Microtubule Cytoskeleton

TL;DR: The functions of the neuronal microtubules cytoskeleton, its architecture, and the way its organization and dynamics are shaped by microtubule-related proteins are highlighted.
Journal ArticleDOI

Genome-wide Analyses Identify KIF5A as a Novel ALS Gene.

Aude Nicolas, +435 more
- 21 Mar 2018 - 
TL;DR: Interestingly, mutations predominantly in the N-terminal motor domain of KIF5A are causative for two neurodegenerative diseases: hereditary spastic paraplegia and Charcot-Marie-Tooth type 2.
References
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Journal ArticleDOI

Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation.

TL;DR: In this article, the authors found that mutations of human Cu,Zn superoxide dismutase (SOD) contribute to the pathogenesis of familial amyotrophic lateral sclerosis (ALS).
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Lysine Acetylation Targets Protein Complexes and Co-Regulates Major Cellular Functions

TL;DR: A proteomic-scale analysis of protein acetylation suggests that it is an important biological regulatory mechanism and the regulatory scope of lysine acetylations is broad and comparable with that of other major posttranslational modifications.
Journal ArticleDOI

Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development

TL;DR: It is concluded that Mfn1 and Mfn2 have both redundant and distinct functions and act in three separate molecular complexes to promote mitochondrial fusion, and by enabling cooperation between mitochondria, has protective effects on the mitochondrial population.
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Conversion of p35 to p25 deregulates Cdk5 activity and promotes neurodegeneration

TL;DR: It is found that p25, a truncated form of p35, accumulates in neurons in the brains of patients with Alzheimer's disease, and this accumulation correlates with an increase in Cdk5 kinase activity.
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