Several mutations and atypical splice variants of WISP3 (CCN6) have been linked to connective tissue disorders and different forms of malignancies. Functional studies have suggested that WISP3 contributes to tissue maintenance/homeostasis. The precise molecular mechanism of WISP3 function in different cell types, however, remains unresolved. The present study was conducted to investigate the potential impact of WISP3 on the accumulation of reactive oxygen species (ROS) and oxidative stress, which are central to cell/tissue maintenance. Our experimental results suggest that WISP3 regulates the accumulation of cellular ROS, and mutations in WISP3 or loss of expression of WISP3 compromise this function.
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http://dx.doi.org/10.1016/j.bbrc.2007.01.114 | DOI Listing |
J Orthop Case Rep
April 2024
Department of Orthopedic and Trauma, Cliniques Universitaires Saint-Luc UCL, UCLouvain, Bruxelles, Belgium.
Ann Rheum Dis
December 2023
Department of Orthopedic Surgery, Boston Children's Hospital, Boston, Massachusetts, USA
Objectives: Progressive pseudorheumatoid arthropathy of childhood (PPAC), caused by deficiency of (), has been challenging to study because no animal model of the disease exists and cartilage recovered from affected patients is indistinguishable from common end-stage osteoarthritis. Therefore, to gain insights into why precocious articular cartilage failure occurs in this disease, we made in vitro derived articular cartilage using isogenic -deficient and -sufficient human pluripotent stem cells (hPSCs).
Methods: We generated articular cartilage-like tissues from induced-(i) PSCs from two patients with PPAC and one wild-type human embryonic stem cell line in which we knocked out WISP3.
Exp Hematol Oncol
July 2023
Department of Oncology, Shanghai Pulmonary Hospital & Thoracic Cancer Institute, Tongji University School of Medicine, Shanghai, China.
Background: Fibronectin, an extracellular matrix protein, has been reported to be associated with heterogeneous cancer stemness, angiogenesis and progression in multiple cancer types. However, the roles and the underlying mechanism of fibronectin on the progression NSCLC need to be further elucidated.
Methods: Public dataset such as Kaplan-Meier Plotter was used to determine the prognostic significance of genes.
Medicine (Baltimore)
July 2023
Department of Clinical Immunology, Xijing Hospital, The Fourth Military Medical University, Xi'an, China.
Introduction: Progressive pseudorheumatoid dysplasia (PPRD) is a rare autosomal recessive genetic disease caused by mutations in the Wnt1-inducible signaling pathway protein 3 gene. PPRD is considered a noninflammatory disease, and involvement of the sacroiliac joint and hip arthritis have not been reported previously.
Patient Concerns: We report a case of PPRD in an 11-year-old boy, who presented with bilateral pain and swelling in the knees, elbows, and ankles, and bilateral pain without swelling in the shoulders, wrists, knuckles, and proximal and distal interphalangeal joints for the past 5 years.
Am J Med Genet A
September 2023
Clinical Genetics Department, Human Genetics and Genome Research Institute, National Research Centre, Cairo, Egypt.
Progressive pseudorheumatoid dysplasia (PPRD), a rare autosomal recessive syndrome, is a type of skeletal dysplasia associated with pain, stiffness, swelling of multiple joints, and the absence of destructive changes. PPRD occurs due to loss of function pathogenic variants in WISP3 (CCN6) gene, located on chromosome 6q22. In this study, 23 unrelated Egyptian PPRD patients were clinically diagnosed based on medical history, physical and radiological examinations, and laboratory investigations.
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