Juan S. Bonifacino

KIF1A-driven entry of degradative lysosomes into the axon is developmentally regulated by the microtubule environment at the axon initial segment

This study shows that KIF1A-mediated lysosomal transport into axons is regulated by the axon initial segment microtubule environment, and its disruption leads to axonal degeneration.

BLOC1S1 variants cause lysosomal and autophagic defects resulting in a hypomyelinating leukodystrophy with epileptic encephalopathy

This study identifies BLOC1S1 variants causing a hypomyelinating leukodystrophy with epileptic encephalopathy, showing impaired lysosomal and autophagic function.

Non-synaptic Mechanism of Ocular Dominance Plasticity

This paper investigates a non-synaptic mechanism of ocular dominance plasticity in adult mice, showing that visual disruption alters spike time arrival and myelin morphology.

Spatial Regulation of Lysosomal Vesicle Acidification Along the Axon via mRAVE-Dependent v-ATPase Assembly

The paper shows that mRAVE-dependent v-ATPase assembly regulates spatial lysosomal acidification in axons by controlling V1-V0 domain association and vesicle transport.

CHIP protects lysosomes from CLN4 mutant-induced membrane damages

The paper identifies CHIP-mediated microautophagy as a protective mechanism against lysosomal damage caused by CLN4 mutations, showing its therapeutic potential in neurodegenerative diseases.

Regulation of angiogenesis by endocytic trafficking mediated by cytoplasmic dynein 1 light intermediate chain 1

This study shows that LIC1 and RILPL1/2 restrict angiogenesis by promoting VEGFR2 degradation, and their disruption increases angiogenesis through enhanced Rab11-mediated recycling.

Programmed ribosomal frameshifting during PLEKHM2 mRNA decoding generates a constitutively active mediator of kinesin-1-dependent lysosome transport

The paper identifies a programmed ribosomal frameshifting event in PLEKHM2 mRNA that generates a constitutively active protein domain essential for lysosome transport and cardiac function.

Mutations in Auxilin cause parkinsonism via impaired clathrin-mediated trafficking at the Golgi apparatus and synapse

This study identifies Auxilin mutations as a cause of Parkinson's disease by showing they impair clathrin-mediated trafficking at the Golgi and synapse, leading to neuropathological lesions.