IFIH1 (Interferon Induced With Helicase C Domain 1)

TBLC enabled accurate differentiation between relapse and fibrotic progression, emphasizing its utility in long-term management. Persistent serologic activity warrants close follow-up, and TBLC may aid in selecting individualized treatment strategies in post-remission deterioration.

Our results show that anti-MDA5+ B cells recognize the helicase domains, which are the enzymatically active domains of the protein. These results have implications for understanding the etiopathology of anti-MDA5+ DM and development of new antigen-specific therapies.

P2, N=108, Not yet recruiting, Intergroupe Francophone de Cancerologie Thoracique

There, it recognizes the degron motifs of RIG-I and MDA5 through its coiled-coil domain and catalyzes their K27-linked ubiquitination and degradation, thereby preventing excessive antiviral signaling. These findings shed light on the significant and dual regulatory roles of RNF20 in maintaining innate immune homeostasis.

Complement activation and the accumulation of M2-like macrophages are associated with anti-human MDA5 monoclonal antibody-induced hepatic injury in mice. These findings provide mechanistic insight into antibody-complement-macrophage interactions and suggest that modulation of complement pathways may represent a potential therapeutic approach to limit liver and systemic involvement in this disorder.

His condition worsened despite high-dose steroids, cyclophosphamide, and plasma exchange; he required ECMO and died after 40 days...He was treated with steroids, rituximab, nintedanib, and mycophenolate...This case series highlights the full clinical spectrum, from pediatric onset to fulminant adult disease. Multidisciplinary care, awareness of atypical presentations, and continued research into disease mechanisms and therapeutics are essential to improving survival in MDA5-DM.

Activation of HSF1 in aged wild-type HSCs partially restores youthful features, supporting a causal role for proteostasis maintenance. Collectively, our findings demonstrate that attenuating MDA5-dependent inflammation preserves HSC function during aging by maintaining metabolic fitness and proteostasis and provide insight into potential therapeutic strategies for mitigating hematopoietic aging.

Loss of IFIH1/MDA5 activity has diverse effects on anti-viral immunity, associated damage and susceptibility to inflammatory disease, but also protection against organ-specific immune-mediated pathology. Findings highlight the importance of IFIH1 in immune regulation and warrant future studies exploring its potential as a diagnostic and therapeutic target.

In vivo, co‑treatment enhanced IFN‑β induction, increased CD8⁺ T‑cell infiltration and reduced tumor burden relative to monotherapies, whereas efficacy was not observed in athymic nude mice, supporting T‑cell dependence. Together, these data provide preclinical evidence that a rational oncolytic virus and STING combination can amplify antitumor immunity without overtly compromising viral persistence.

Our study revealed that DNA damage response altered the tumor structure and modulated the expression of multiple genes, including extracellular matrix- and immune-related genes. Studies using in vitro organoid models can be useful for investigating three-dimensional cellular responses after DNA-damaging cancer therapies, such as chemoradiotherapy.

A 74-year-old Japanese woman with rapidly progressive DM-associated ILD was treated with high-dose glucocorticoids, intravenous cyclophosphamide, and cyclosporine, followed by tofacitinib due to an insufficient response. Discontinuation of tofacitinib alone resulted in rapid clinical and radiological improvement. This case highlights PAP as a rare, potentially reversible complication during JAK inhibitor therapy in anti-MDA5 antibody-positive DM-associated ILD and underscores the importance of considering PAP when elevated KL-6 levels and radiological abnormalities occur despite only mild respiratory symptoms.

The results suggest that MDA5 participates in the inflammation of pulps following caries. In addition, it may be hypothesized that MDA5 expression can be sex-dependent in the inflamed pulps.