The Charcot-Marie-Tooth disease 2F (CMT2F) and distal hereditary motor neuropathy 2B (dHMN2B) are caused by autosomal dominantly inherited mutations of the heat shock 27 kDa protein 1 (HSPB1) gene and there are no specific therapies available yet. Here, we assessed the potential therapeutic effect of HDAC6 inhibitors on peripheral neuropathy with HSPB1 mutation using in vitro model of motor neu...

Hermansky-Pudlak syndrome 6 protein (HPS6) has originally been identified as a subunit of the BLOC-2 protein complex that is involved in the biogenesis of lysosome-related organelles. Here, we demonstrate that HPS6 directly interacts with the dynactin p150 subunit of the dynein–dynactin motor complex and acts as cargo adaptor for the retrograde motor to mediate the transport of lysosomes from t...

The human primary motor cortex (M1) is robustly activated during visually guided hand movements. M1 multivoxel patterns of functional MRI activation are more correlated during repeated hand movements to the same targets than to greatly differing ones, and therefore potentially contain information about movement direction. It is unclear, however, whether direction specificity is due to the motor...

Movement-related potentials (MRPs) associated with voluntary movements reflect cortical activity associated with processes of movement preparation and movement execution. Early-stage pre-movement activity is reduced in amplitude in Parkinson's disease. However, it is unclear whether this neurophysiological deficit relates to preparatory or execution-related activity, since previous studies have...

Rab GTPases and their effectors regulate membrane traffic by determining, along with cognate SNAREs, the specificity of transport vesicle docking and fusion steps. Recent studies have also implicated Rabs in the movement of these transport vesicles from their site of formation to their site of fusion, and several Rabs have been linked to specific microtubuleor actin-based motor proteins. Analys...

Primary motor cortex has been studied for more than a century, yet a consensus on its functional contribution to movement control is still out of reach. In particular, there remains controversy as to the level of control produced by motor cortex ("low-level" movement dynamics vs. "high-level" movement kinematics) and the role of sensory feedback. In this review, we present different perspective...

A recently emerging view sees language understanding as closely linked to sensory and motor processes. The present study investigates this issue by examining the influence of processing action verbs and concrete nouns on the execution of a reaching movement. Fine-grained analyses of movement kinematics revealed that relative to nouns, processing action verbs significantly affect overt motor per...

Previous studies have suggested that complex movements can be elicited by electrical stimulation of the motor cortex. Most recording studies in the motor cortex, however, have investigated the encoding of time-independent features of movement such as direction, velocity, position, or force. Here, we show that single motor cortical neurons encode temporally evolving movement trajectories and not...