Disruption of axonal transport in neurodegeneration

SH Berth, TE Lloyd - The Journal of Clinical Investigation, 2023 - Am Soc Clin Investig
Neurons are markedly compartmentalized, which makes them reliant on axonal transport to
maintain their health. Axonal transport is important for anterograde delivery of newly …

Pluripotent stem cells in disease modelling and drug discovery

Y Avior, I Sagi, N Benvenisty - Nature reviews Molecular cell biology, 2016 - nature.com
Experimental modelling of human disorders enables the definition of the cellular and
molecular mechanisms underlying diseases and the development of therapies for treating …

Hereditary spastic paraplegia: from diagnosis to emerging therapeutic approaches

S Shribman, E Reid, AH Crosby, H Houlden… - The Lancet …, 2019 - thelancet.com
Hereditary spastic paraplegia (HSP) describes a heterogeneous group of genetic
neurodegenerative diseases characterised by progressive spasticity of the lower limbs. The …

Myelin damage and repair in pathologic CNS: challenges and prospects

A Alizadeh, SM Dyck… - Frontiers in molecular …, 2015 - frontiersin.org
Injury to the central nervous system (CNS) results in oligodendrocyte cell death and
progressive demyelination. Demyelinated axons undergo considerable physiological …

Microtubule-severing enzymes: From cellular functions to molecular mechanism

FJ McNally, A Roll-Mecak - Journal of Cell Biology, 2018 - rupress.org
Microtubule-severing enzymes generate internal breaks in microtubules. They are
conserved in eukaryotes from ciliates to mammals, and their function is important in diverse …

Axonal endoplasmic reticulum dynamics and its roles in neurodegeneration

Z Öztürk, CJ O'Kane, JJ Pérez-Moreno - Frontiers in neuroscience, 2020 - frontiersin.org
The physical continuity of axons over long cellular distances poses challenges for their
maintenance. One organelle that faces this challenge is endoplasmic reticulum (ER); unlike …

Hereditary spastic paraplegia

C Blackstone - Handbook of clinical neurology, 2018 - Elsevier
The hereditary spastic paraplegias (HSPs) are a heterogeneous group of neurologic
disorders with the common feature of prominent lower-extremity spasticity, resulting from a …

Defects in ER–endosome contacts impact lysosome function in hereditary spastic paraplegia

R Allison, JR Edgar, G Pearson, T Rizo… - Journal of Cell …, 2017 - rupress.org
Contacts between endosomes and the endoplasmic reticulum (ER) promote endosomal
tubule fission, but the mechanisms involved and consequences of tubule fission failure are …

Hereditary spastic paraplegia SPG4: what is known and not known about the disease

JM Solowska, PW Baas - Brain, 2015 - academic.oup.com
Mutations in more than 70 distinct loci and more than 50 mutated gene products have been
identified in patients with hereditary spastic paraplegias, a diverse group of neurological …

Pervasive axonal transport deficits in multiple sclerosis models

CD Sorbara, NE Wagner, A Ladwig, I Nikić, D Merkler… - Neuron, 2014 - cell.com
Impaired axonal transport can contribute to axon degeneration and has been described in
many neurodegenerative diseases. Multiple sclerosis (MS) is a common neuroinflammatory …