Andreas Hermann

RIG-I mediated neuron-specific IFN type 1 signaling in FUS-ALS induces neurodegeneration and offers new biomarker-driven individualized treatment options for (FUS-)ALS

The paper identifies RIG-I-mediated innate immune activation in FUS-ALS spinal motor neurons due to mitochondrial dsRNA accumulation, which can be targeted by JAK-STAT inhibition, offering potential biomarker-driven treatment options.

ALS-Linked FUS and SOD1 Mutations Elevate MCM2 in Human Motor Neurons

This study shows that ALS-linked FUS and SOD1 mutations increase MCM2 protein levels in human motor neurons without affecting its subcellular localization.

HMGB1 B-box domain self-complexes promote protein-polyelectrolyte interactions.

This study shows that HMGB1's B-box domain forms self-associates that enhance interactions with heparin through charge redistribution and conformational changes.

Small molecule inhibitors of hnRNPA2B1-RNA interactions reveal a predictable sorting of RNA subsets into extracellular vesicles

This study identifies small molecule inhibitors of hnRNPA2B1-RNA interactions and shows they alter EV-RNA composition and paracrine signaling.

Axonal transcriptome reveals upregulation of PLK1 as a protective mechanism in response to increased DNA damage in FUSP525L spinal motor neurons

The paper uses compartment-specific RNA-sequencing to analyze axonal and somatodendritic transcriptomes of FUS-ALS motor neurons, identifying PLK1 upregulation as a protective response to DNA damage.

PP2A and GSK3 act as modifiers of FUS-ALS by modulating mitochondrial transport

This study identifies PP2A and GSK3 as modifiers of FUS-ALS by modulating mitochondrial transport in Drosophila and human iPSC-derived motor neurons.

DJ-1 products glycolic acid and D-lactate restore deficient axonal trafficking and DNA damage response in FUS and SOD1-associated amyotrophic lateral sclerosis

The paper shows that glycolic acid and D-lactate restore axonal trafficking and DNA damage response in FUS and SOD1-ALS patient-derived motoneurons.

Live cell imaging of ATP levels reveals metabolic compartmentalization within motoneurons and early metabolic changes in FUS ALS motoneurons.

The paper uses live cell imaging to show reduced ATP levels in FUS-ALS motoneurons, linking metabolic changes to disease vulnerability.

High content live profiling reveals concomitant gain and loss of function pathomechanisms in C9ORF72 amyotrophic lateral sclerosis.

This study shows that C9ORF72 ALS pathogenesis involves both gain and loss of function mechanisms causing axonal organelle trafficking impairment and DNA damage.

Age-dependent neurodegeneration and organelle transport deficiencies in mutant TDP43 patient-derived neurons are independent of TDP43 aggregation

This study shows that TDP43 mutations cause motor neuron degeneration in ALS through mechanisms unrelated to TDP43 aggregation or mislocalization.