Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1165/rcmb.2023-0084LE | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
A histochemical approach to activity-based copper sensing reveals cuproplasia-dependent vulnerabilities in cancer.
Proc Natl Acad Sci U S A
January 2025
Department of Chemistry, University of California, Berkeley, CA 94720.
Copper is an essential nutrient for sustaining vital cellular processes spanning respiration, metabolism, and proliferation. However, loss of copper homeostasis, particularly misregulation of loosely bound copper ions which are defined as the labile copper pool, occurs in major diseases such as cancer, where tumor growth and metastasis have a heightened requirement for this metal. To help decipher the role of copper in the etiology of cancer, we report a histochemical activity-based sensing approach that enables systematic, high-throughput profiling of labile copper status across many cell lines in parallel.
View Article and Find Full Text PDFMutations disrupting the kinase domain of IKKα lead to immunodeficiency and immune dysregulation in humans.
J Exp Med
February 2025
Laboratory of Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, University Paris Cité, Paris, France.
IKKα, encoded by CHUK, is crucial in the non-canonical NF-κB pathway and part of the IKK complex activating the canonical pathway alongside IKKβ. The absence of IKKα causes fetal encasement syndrome in humans, fatal in utero, while an impaired IKKα-NIK interaction was reported in a single patient and causes combined immunodeficiency. Here, we describe compound heterozygous variants in the kinase domain of IKKα in a female patient with hypogammaglobulinemia, recurrent lung infections, and Hay-Wells syndrome-like features.
View Article and Find Full Text PDFEndocytic recycling is central to circadian collagen fibrillogenesis and disrupted in fibrosis.
Elife
January 2025
Wellcome Centre for Cell-Matrix Research, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, United Kingdom.
Collagen-I fibrillogenesis is crucial to health and development, where dysregulation is a hallmark of fibroproliferative diseases. Here, we show that collagen-I fibril assembly required a functional endocytic system that recycles collagen-I to assemble new fibrils. Endogenous collagen production was not required for fibrillogenesis if exogenous collagen was available, but the circadian-regulated vacuolar protein sorting (VPS) 33b and collagen-binding integrin α11 subunit were crucial to fibrillogenesis.
View Article and Find Full Text PDFPulmonary Arterial Hypertension in a Patient With a Lung Mass: Any Link?
Cureus
December 2024
Pulmonology, King Abdulaziz Medical City, Jeddah, SAU.
A 52-year-old female patient with a history of atrial septal defect repair presented with progressive dyspnea and echocardiographic findings suggestive of pulmonary hypertension (PH). Incidentally, a lung mass was discovered on computed tomography (CT). Initial evaluation revealed World Health Organization functional class III symptoms and significant weight loss.
View Article and Find Full Text PDFThe Clinical Laboratory (CL) is involved in the prevention, diagnosis and follow-up of disease, as well as in the monitoring of treatment. For this reason, the CL must have robust quality systems in place in order to provide reliable results that help to ensure correct health care. Since the entry into force of the European regulation (IVDR) on in vitro diagnostic medical devices (EU) 2017/746 has generated the loss of CE marking in some laboratory determinations.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!