0000000000890211
AUTHOR
Manuel Mata Roig
Primary ciliary dyskinesia. Ciliopathies
Abstract Primary ciliary dyskinesia is a genetically inherited syndrome characterised by ciliary immotility or dysmotility. Deficiency in mucociliary clearance produces chronic respiratory infections from birth, male sterility by spermatozoid immotility and situs inversus in 40%–50% of patients (Kartagener's syndrome). Diagnosis is made by analysing ciliary motility with high-speed digital video and ciliary ultrastructure. The wide distribution and functions of the cilia in the body mean that this dysfunction can generate other ciliopathies apart from primary ciliary dyskinesia.
Cartilage Regeneration and Tissue Engineering
Abstract Articular cartilage in synovial joints is a hyaline cartilage highly hydrated with a rigorous order of cells and fibers and a specific content of proteoglycans and glycoproteins. It provides a low-friction surface, participates in the lubrication of the synovial joints, and distributes the forces to the underlying bone. It is an avascular and aneural tissue where small metabolites diffuse to and from cells. Unlike hyaline cartilage in other locations, articular cartilage lacks perichondrium, a layer of fibrous tissue around it that serves as the source of new cartilage cells. Thus, although extracellular matrix undergoes continuous remodeling throughout life, the ability to repair …