bims-actimu Biomed News
on Actinopathies in inborn errors of immunity
Issue of 2025–04–06
three papers selected by
Elodie Busch, University of Strasbourg



  1. J Hematop. 2025 Apr 02. 18(1): 13
      A 20-year-old man with a history of Wiskott-Aldrich syndrome (WAS) underwent excisional biopsy of a femoral lymph node for evaluation of lymphadenopathy. Histologic examination showed paracortical expansion by a lymphohistiocytic infiltrate, and reactive follicles with regressive changes. The interfollicular histiocytes had clumped nuclei and showed occasional emperipolesis. Special stains were negative for infectious organisms. The microscopic findings in this lymph node are related to the underlying WAS. Emperipolesis has previously been reported in lymph nodes of patients with WAS. Recognition of reactive microscopic features in lymph nodes of these patients is important, as they are at increased risk for lymphoproliferative disorders.
    Keywords:  Emperipolesis; Lymph node; Lymphadenopathy; Pathology; Wiskott-Aldrich syndrome
    DOI:  https://doi.org/10.1007/s12308-025-00628-8
  2. Biochim Biophys Acta Rev Cancer. 2025 Mar 26. pii: S0304-419X(25)00046-0. [Epub ahead of print]1880(3): 189304
      The Coronin family, a class of actin-binding proteins involved in the formation and maintenance of cytoskeleton structural stability, is aberrantly expressed in various tumors, including lung, gastric and head and neck cancers. They can regulate tumor cell metabolism and proliferation through RAC-1 and Wnt/β-Catenin signaling pathways and regulate invasion by influencing the PI3K, PAK4, and MT1-MMP signaling pathways and impacting the actin-network dynamics. In recent years, an increasing number of studies have highlighted the crucial roles of the cytoskeleton and immune modulation in the occurrence and development of tumors. The article delves into the Coronin family's pivotal role in tumor immune evasion, highlighting its modulation of neutrophil, T cell, and vesicular transport functions, as well as its interactions with tumorigenesis related organelles such as the endoplasmic reticulum, Golgi apparatus, mitochondria, and lysosomes. It also summarizes the potential therapeutic applications of the Coronin family in oncology. This review provides valuable insights into the mechanisms through which the Coronin family is implicated in the onset and progression of tumors. It also provides more theoretical foundation for tumor immunotherapy and combination drug therapy.
    Keywords:  Cellular organelles; Coronin family; Cytoskeleton; Immunotherapy; Tumorigenesis
    DOI:  https://doi.org/10.1016/j.bbcan.2025.189304
  3. Redox Biol. 2025 Mar 26. pii: S2213-2317(25)00131-4. [Epub ahead of print]82 103618
      The double-edged role of neutrophils in effective host defense and harmful pathology is an emerging topic in clinical research. Neutrophils release highly potent antimicrobial granule compounds and reactive oxygen species (ROS) that can also be detrimental to the host and promote inflammatory diseases and cancer. Here we show that disassembly of F-actin greatly facilitates ROS production and degranulation in neutrophils. Utilizing neutrophils from Coronin 1a (Coro1a)-deficient mice, our data reveal that the actin-regulatory protein Coro1a controls this spatial F-actin deconstruction and concomitantly forms a signaling complex with Rac-GTPases, thereby promoting activation and translocation of Rac to the membrane during neutrophil activation. This functional activity of Coro1a was critical for neutrophil granule exocytosis and the activation of the NADPH oxidase complex. Consistent with these findings, impaired ROS production in Coro1a-deficient neutrophils was rescued by pharmacological promotion of actin depolymerization or activation of Rac. Together, our findings suggest that the Coro1a/Rac signaling hub acts as a central regulatory element that coordinates actin cytoskeletal reorganization required for the execution of neutrophil effector functions. Since Coro1a is highly conserved between mice and humans and associated with human immunodeficiency, our results are also relevant for human biomedical studies.
    Keywords:  Actin cytoskeleton; Coronin1a; NADPH oxidase; Neutrophil effector function; Oxidative burst
    DOI:  https://doi.org/10.1016/j.redox.2025.103618