Robert Gordon Kalb

Ubiquitin Proteasome System Components, RAD23A and USP13, Modulate TDP-43 Solubility and Neuronal Toxicity

This study identifies RAD23A and USP13 as modifier genes that enhance TDP-43 solubility and reduce neuronal toxicity in ALS models.

The molecular mechanism of uptake and cell-to-cell transmission of arginine-containing dipeptide repeat proteins.

This paper identifies CSPG4 as a mediator of arginine-rich DPR uptake and cell-to-cell transmission, suggesting it as a therapeutic target for ALS/FTD.

A role for the spinal cord cholinergic neuron circadian clock in RNA metabolism and mediating ALS disease phenotypes

The paper shows that the circadian clock in spinal cord cholinergic neurons affects RNA metabolism and contributes to ALS disease phenotypes through rhythmic gene expression and alternative splicing.

Reduction of RAD23A extends lifespan and mitigates pathology in TDP-43 mice

This study shows that reducing RAD23A in TDP-43 mice improves survival, behavior, and reduces TDP-43 pathology and transcriptomic alterations.

The nuclear ubiquitin ligase adaptor SPOP is a conserved regulator of C9orf72 dipeptide toxicity

This paper identifies SPOP as a conserved regulator of C9orf72 dipeptide toxicity in C. elegans and mammalian neurons, showing SPOP inhibition protects against toxicity.

Neurons undergo pathogenic metabolic reprograming in models of familial ALS

The paper identifies metabolic reprogramming in neurons of familial ALS models, showing impaired glycolytic flux with maintained citric acid cycle activity and normal ATP levels.

Preconditioning of Caenorhabditis elegans to Anoxic Insult by Inactivation of Cholinergic, GABAergic, and Muscle Activity

This study shows that inactivating the nervous system and muscles in C. elegans before an anoxic insult increases resistance to prolonged anoxia.