The Role of Sclerostin in Bone and Ectopic Calcification [PDF]
Sclerostin, a 22-kDa glycoprotein that is mainly secreted by the osteocytes, is a soluble inhibitor of canonical Wnt signaling. Therefore, when present at increased concentrations, it leads to an increased bone resorption and decreased bone formation. Serum sclerostin levels are known to be increased in the elderly and in patients with chronic kidney ...
Annelies De Maré +2 more
exaly +6 more sources
P2X7 Purinoceptor Affects Ectopic Calcification of Dystrophic Muscles [PDF]
Ectopic calcification (EC) of myofibers is a pathological feature of muscle damage in Duchenne muscular dystrophy (DMD). Mineralisation of muscle tissue occurs concomitantly with macrophage infiltration, suggesting a link between ectopic mineral ...
Robin M. H. Rumney +6 more
doaj +6 more sources
Anticalcification effects of DS-1211 in pseudoxanthoma elasticum mouse models and the role of tissue-nonspecific alkaline phosphatase in ABCC6-deficient ectopic calcification [PDF]
Pseudoxanthoma elasticum (PXE) is a multisystem, genetic, ectopic mineralization disorder with no effective treatment. Inhibition of tissue-nonspecific alkaline phosphatase (TNAP) may prevent ectopic soft tissue calcification by increasing endogenous ...
Kaori Soma +2 more
doaj +2 more sources
Ectopic Calcification: What Do We Know and What Is the Way Forward? [PDF]
Ectopic calcification, or ectopic mineralization, is a pathologic condition in which calcifications develop in soft tissues [...]
Snijders BMG, Peters MJL, Koek HL.
europepmc +3 more sources
Liver-directed base editing of ABCC6 prevents ectopic calcification in a variant-humanized mouse model of pseudoxanthoma elasticum [PDF]
Pseudoxanthoma elasticum (PXE) is an autosomal recessive connective tissue disorder characterized by ectopic calcification of elastic fibers throughout the skin, retina, and arteries.
Lauren C. Testa +8 more
doaj +2 more sources
ENPP1 variants in patients with GACI and PXE expand the clinical and genetic heterogeneity of heritable disorders of ectopic calcification. [PDF]
Pseudoxanthoma elasticum (PXE) and generalized arterial calcification of infancy (GACI) are clinically distinct genetic entities of ectopic calcification associated with differentially reduced circulating levels of inorganic pyrophosphate (PPi), a potent
Douglas Ralph +11 more
doaj +2 more sources
Activation of Nuclear Factor Erythroid 2-Related Factor 2 Transcriptionally Upregulates Ectonucleotide Pyrophosphatase/Phosphodiesterase 1 Expression and Inhibits Ectopic Calcification in Mice [PDF]
Calcification plays a key role in biological processes, and breakdown of the regulatory mechanism results in a pathological state such as ectopic calcification.
Ida Tomomi +9 more
doaj +2 more sources
Angiogenesis and Pericytes in the Initiation of Ectopic Calcification [PDF]
Ectopic calcification of blood vessels, heart valves, and skeletal muscle is a major clinical problem. There is now good evidence that angiogenesis is associated with ectopic calcification in these tissues and that it is necessary, but not sufficient, for calcification to occur. Angiogenesis may regulate ectopic calcification in several ways.
Ann E Canfield
exaly +4 more sources
ABCC6, Pyrophosphate and Ectopic Calcification: Therapeutic Solutions. [PDF]
Pathological (ectopic) mineralization of soft tissues occurs during aging, in several common conditions such as diabetes, hypercholesterolemia, and renal failure and in certain genetic disorders. Pseudoxanthoma elasticum (PXE), a multi-organ disease affecting dermal, ocular, and cardiovascular tissues, is a model for ectopic mineralization disorders ...
Shimada BK +5 more
europepmc +4 more sources
Gitelman syndrome and ectopic calcification in the retina and joints. [PDF]
Abstract Gitelman syndrome is a rare inherited renal tubular disorder with features that resemble thiazide use, including a hypokalemic metabolic alkalosis, hypomagnesemia, hypocalciuria and a low or normal blood pressure, hyperreninemia and hyperaldosteronism. Treatment is primarily correction of the potassium and magnesium levels.
Ham Y +4 more
europepmc +5 more sources

