Results 61 to 70 of about 8,705 (254)
Matrix Gla Protein involved in elastic fiber calcification in the dermis of pseudoxanthoma elasticum patients. [PDF]
Mature MGP (Matrix gamma-carboxyglutamic acid protein) is known to inhibit soft connective tissues calcification. We investigated its possible involvement in pseudoxanthoma elasticum (PXE), a genetic disorder whose clinical manifestations are due to ...
ANNOVI, Giulia +7 more
core +1 more source
Pseudoxanthoma elasticum (PXE), the prototype of heritable ectopic mineralization disorders, manifests with deposition of calcium hydroxyapatite crystals in the skin, eyes and arterial blood vessels. This autosomal recessive disorder, due to mutations in
Hongbin Luo, Qiaoli Li, Yi Cao, J. Uitto
semanticscholar +1 more source
Importance Correlates for Bruch membrane alterations are needed for interventional trials targeting the Bruch membrane in pseudoxanthoma elasticum (PXE).
Kristina Hess +8 more
semanticscholar +1 more source
Rate of change of carotid intima-media thickness with magnesium administration in Abcc6⁻/⁻ mice. [PDF]
Pseudoxanthoma elasticum (PXE), caused by mutations in the ABCC6 gene, demonstrates progressive build-up of calcium phosphate and proteoglycans in the skin, eye, and arteries, and is associated to myocardial infarctions, stroke, blindness, and elevated ...
Boutouyrie +10 more
core +1 more source
Induction of diabetes in three different mouse strains uniformly resulted in an increase in TNAP activity and a reduction in pyrophosphate (PPi) in the circulation. Inhibition of TNAP restored plasma PPi. Diabetes‐induced calcification in the media layer of the aorta was detected only in the Abcc6−/− strain, which is predisposed to ectopic ...
Krisztina Fülöp +13 more
wiley +1 more source
Pseudoxanthoma elasticum and reflectance confocal microscopy: report of two affected young sisters [PDF]
Pseudoxanthoma elasticum (PXE) is a rare inherited multisystem disorder that mainly affects skin, eyes and cardiovascular system. The associated clinical signs are due to progressive calcification of elastic fibres and blood vessels, despite normal ...
BORALDI, Federica +5 more
core +3 more sources
The extent of angioid streaks correlates with macular degeneration in pseudoxanthoma elasticum.
PURPOSE To investigate whether the extent of Bruch's membrane (BM) calcification is associated with choroidal neovascularization (CNV) and macular atrophy in patients with pseudoxanthoma elasticum (PXE) by using the extent of angioid streaks as a ...
S. Risseeuw +5 more
semanticscholar +1 more source
Angioid streaks. A case report
Angioid streaks are breaks in Bruch's membrane displayed at the bottom of the eye as orange or gray bands around the optic disc, and from that point on they extend radially.
Aimé Broche Hernández +2 more
doaj +2 more sources
Gram‐Scale Synthesis of an Arylsulfonamide‐Type Alkaline Phosphatase Inhibitor
A larger amount of a tissue‐nonspecific alkaline phosphatase inhibitor, 5‐((5‐chloro‐2‐methoxyphenyl)sulfonamido)nicotinamide, was synthesized in five steps using a parallel method without the need for chromatography or crystallization. Because a single batch was used for in vivo studies in a large population of mice, the pharmacological results were ...
Zsolt Rapi +2 more
wiley +1 more source
Oral administration of pyrophosphate inhibits connective tissue calcification [PDF]
Various disorders including pseudoxanthoma elasticum (PXE) and generalized arterial calcification of infancy (GACI), which are caused by inactivating mutations in ABCC6 and ENPP1, respectively, present with extensive tissue calcification due to reduced ...
Arányi, Tamás +5 more
core +1 more source

