James N. Sleigh

The paper investigates a dual-strategy approach using a muscle-tropic AAV capsid and muscle-specific promoter to improve gene delivery efficiency and reduce off-target expression in skeletal muscle.

This study shows that the Gars{Delta}ETAQ mutation in mice causes specific peripheral nerve pathology in motor and sensory neurons, affecting multiple functions and providing a model for CMT2D research.

The paper identifies a sex-specific neural memory mechanism in the pancreas that influences epithelial plasticity and tissue resilience after injury.

This study shows BDNF enhances axonal regeneration in human motor neurons by regulating RNA metabolism and cytoskeletal dynamics through kinase pathways.

The paper shows TBK1 phosphorylates Rab7 to regulate unidirectional retrograde transport of signalling endosomes in motor neurons, linking TBK1 dysfunction to ALS-related transport impairments.

This study shows that CNTF specifically slows axonal transport of signalling endosomes in both fast and slow motor neurons, highlighting selectivity in NTF regulation of transport.

This study identifies sex-dependent motor neuron dysfunction and cargo-specific axonal transport impairments in FUS-ALS mice, linking endosomal transport defects to denervation in ALS.

The paper investigates how aging affects signaling endosome transport in motor axons and finds that processivity is impaired, which is not rescued by BDNF treatment due to reduced TrkB receptor expression.

The paper investigates axonal transport dynamics of signalling endosomes and mitochondria at the Node of Ranvier in vivo, finding reduced velocity and increased pausing during transit through the NoR.

This study investigates age-related changes in mitochondrial function and cytoplasmic viscosity in mouse peripheral neurons, finding compartment-specific effects on mitochondrial trafficking and homeostasis.

This study shows that BDNF-dependent axonal transport is selectively impaired in fast motor neurons of ALS mice, contributing to disease pathology.

NMJ-Analyser quantifies neuromuscular junction morphology and innervation status in mouse models, identifying structural changes in ALS and peripheral neuropathy with high accuracy.

This study examines neuromuscular junction (NMJ) pathology in CMT2D mice, finding a distal-to-proximal denervation gradient and identifying post-natal synaptic growth as a factor in neurodegeneration.

This study quantifies post-synaptic NMJ morphology in different mouse muscles and finds it correlates with fibre type, not diameter, and shows NMJ growth rates vary across muscles.

This study shows that loss of BICD2 in muscle drives motor neuron loss in SMALED2 through a non-cell autonomous mechanism involving impaired secretory pathways.

The paper examines axonal transport defects in two ALS mouse models, finding that TDP-43 mutations cause transport issues while FUS mutations do not.

The paper shows that mutant GlyRS disrupts Nrp1/VEGF-A signaling but does not affect vascular development or maintenance in CMT2D mice.