Kathleen M Cunningham, Kirstin Maulding ... Thomas E Lloyd
Impaired nuclear import of the transcription factor TFEB/MITF is a major cause of autophagy and lysosome dysfunction in amyotrophic lateral sclerosis caused by mutations in the C9orf72 gene.
Nicola S Orefice, Owein Guillemot-Legris ... Giulio G Muccioli
A novel EAE model featuring cortical lesions and an autoimmune heterogeneity, two key parameters missing in the classical model, allows for insights in disease pathogenesis.
Alteration of the G-quadruplex structure formed by GGGGCC repeat RNA through the direct interaction with RNA-binding proteins can suppress pathogenic repeat-associated non-AUG translation, leading to therapeutic effects on neurodegeneration in C9orf72-linked amyotrophic lateral sclerosis and frontotemporal dementia.
A link between chronic stress and organ dysfunction is explained by the gateway reflex, in which brain micro-inflammation at specific vessels establishes a new neural pathway to induce fatal organ failure particularly in gastrointestine and heart.
Zfp106 functions as an RNA binding protein, binds directly to GGGGCC RNA repeats, is required in motor neurons to prevent ALS-like neurodegeneration in mice, and can suppress neurotoxicity in an established fly model of ALS.
Takemichi Fukasawa, Ayumi Yoshizaki ... Shinichi Sato
Single-cell analysis revealed that autoantigen-reactive B cells changed the type of cytokines they produced from anti-inflammatory IL-10 and IL-35 to pro-inflammatory IL-6 and IL-23 as affinity to autoantigen increased.
Ultrastructural and loss-of-function experiments show that oligodendrocyte-encoded Kir4.1 is located near active axonal structures, including within myelin inner tongue, and has critical functions to promote axonal activity and preserve integrity.
