Hong Joo Kim

Competing Molecular Interactions Govern the Dynamical Arrest of G3BP1 Condensates

This study shows that electrostatic interactions between intrinsically disordered regions drive G3BP1 condensate ageing, with RNA modulating these interactions and delaying dynamic arrest.

Alphaviral nonstructural protein-host RBP co-condensation as a mechanism to sustain virus replication

This study identifies nsP3-RBP co-condensation as a mechanism alphaviruses use to sustain replication, showing disruption of these condensates can inhibit viral replication.

Preclinical evaluation of antisense oligonucleotide therapy in a mouse model of HNRNPH2-related neurodevelopmental disorder

This study tests antisense oligonucleotides targeting Hnrnph2 in a mouse model, showing reduced Hnrnph2 levels, increased Hnrnph1, and improved phenotypes like seizures and motor function.

Integrative multiomic analysis links TDP-43-driven splicing defects to cascading proteomic disruption of ALS/FTD pathways

The paper identifies TDP-43-dependent molecular cascades in ALS/FTD by integrating multiomic data from iPSC-derived neurons and validates key genes through experimental assays.

A point mutation in the nuclear speckle protein and splicing factor SRRM2 is associated with amyotrophic lateral sclerosis and causes dysregulation of synapse-associated genes

This study identifies a point mutation in SRRM2 as a risk factor for ALS, showing it disrupts protein interactions and dysregulates synapse-related genes.

Specific heterozygous frameshift variants in hnRNPA2B1 cause early-onset oculopharyngeal muscular dystrophy

The paper identifies heterozygous frameshift variants in hnRNPA2B1 causing early-onset oculopharyngeal muscular dystrophy by altering nucleocytoplasmic transport.

A murine model of hnRNPH2-related neurodevelopmental disorder recapitulates clinical features of human disease and reveals a mechanism for genetic compensation of HNRNPH2

This paper creates a mouse model of HNRNPH2-related neurodevelopmental disorder, showing similar symptoms to humans and revealing genetic compensation mechanisms.

DEFINING THE DIVERSITY OF HNRNPA1 MUTATIONS IN CLINICAL PHENOTYPE AND PATHOMECHANISM

The paper characterizes HNRNPA1 mutations in patients with neurodegenerative diseases, showing varied effects on fibrillization, phase separation, and stress granule dynamics.

OptoGranules reveal the evolution of stress granules to ALS-FTD pathology

The paper introduces OptoGranules, a light-inducible system to control stress granule dynamics in cells, showing that persistent stress granule assembly leads to ALS-FTD-like pathology.