Results 91 to 100 of about 2,584 (187)

Pseudoxanthoma elasticum-associated angioid streaks near a scleral buckle

American Journal of Ophthalmology Case Reports
Purpose: We report a patient with pseudoxanthoma elasticum (PXE) with angioid streaks near a scleral buckle site. Observations: A 46-year-old male with PXE presented for evaluation of blurry vision and was found to have classic PXE findings in both eyes ...
Karen E. Lee, Tobin B.T. Thuma, Mirataollah Salabati, Meera D. Sivalingam, Jose S Pulido, Kammi B. Gunton   +5 more
doaj   +1 more source

Réinstallation de postes clients avec PXE et Partimage

, 2007
Le métier d'administrateur système demande de gérer un maximum de machines avec un minimum d'efforts. Pour ce faire, nous avons cherché une solution de réinstallation automatique d'ordinateurs personnels dual-boot (Windows, Linux) afin d'alléger le ...
Borghesi, Guilhem, Boccheciampe, Christophe   +1 more
core   +3 more sources

Pseudoxanthoma elasticum: cardiac findings in patients and Abcc6-deficient mouse model.

PLoS ONE, 2013
BackgroundPseudoxanthoma elasticum (PXE), caused by mutations in the ABCC6 gene, is a rare multiorgan disease characterized by the mineralization and fragmentation of elastic fibers in connective tissue.
Fabrice Prunier, Gwenola Terrien, Yannick Le Corre, Ailea L Y Apana, Loïc Bière, Gilles Kauffenstein, Alain Furber, Arthur A B Bergen, Theo G M F Gorgels, Olivier Le Saux, Georges Leftheriotis, Ludovic Martin   +11 more
doaj   +1 more source

Novel treatment for PXE: Recombinant ENPP1 enzyme therapy

Molecular Therapy
Pseudoxanthoma elasticum (PXE) is a genetic multisystem ectopic calcification disorder caused by inactivating mutations in the ABCC6 gene encoding ABCC6, a hepatic efflux transporter. ABCC6-mediated ATP secretion by the liver is the main source of a potent endogenous calcification inhibitor, plasma inorganic pyrophosphate (PPi); the deficiency of ...
Ida Joely Jacobs, Dora Obiri-Yeboah, Paul R. Stabach, Demetrios T. Braddock, Qiaoli Li   +4 more
openaire   +3 more sources

Large-scaled metabolic profiling of human dermal fibroblasts derived from pseudoxanthoma elasticum patients and healthy controls.

PLoS ONE, 2014
Mutations in the ABC transporter ABCC6 were recently identified as cause of Pseudoxanthoma elasticum (PXE), a rare genetic disorder characterized by progressive mineralization of elastic fibers.
Patricia Kuzaj, Joachim Kuhn, Ryan D Michalek, Edward D Karoly, Isabel Faust, Mareike Dabisch-Ruthe, Cornelius Knabbe, Doris Hendig   +7 more
doaj   +1 more source

UEG Week 2025 Moderated Posters

United European Gastroenterology Journal, Volume 13, Issue S8, Page S189-S802, October 2025.
wiley   +1 more source

Автоматизація розгортання робочих вузлів обчислювального кластера та їх подальше адміністрування з застосуванням технологій PXE та ANSIBLE

, 2023
Мета роботи – відтворення існуючої у науковій літературі моделі розгортання робочих вузлів обчислювального кластера з допомогою PXE віддалено від інстальованого комп’ютера без використання фізичних носіїв інформації для інсталювання операційної системи ...
Кучер Денис
core  

Novel Human Induced Pluripotent Stem Cell-Based Model for Retinal Pigment Epithelial Cells to Reveal Possible Disease Mechanisms for Macular Degeneration in Pseudoxanthoma Elasticum

Journal of Ophthalmology
Pseudoxanthoma elasticum (PXE) is a rare metabolic disease with autosomal recessive inheritance. The manifestation in PXE is represented by retinal complications, pseudoxanthomas of the skin folding areas, and arterial calcification.
Taina Viheriälä, Heidi Hongisto, Lyydia Saari, Marika Oksanen, Tanja Ilmarinen, Suvi Väärämäki, Hannu Uusitalo, Pasi Nevalainen, Heli Skottman   +8 more
doaj   +1 more source

Radar Cross Section Measurements of Artillery shells

, 2014
In this paper, different RCS measurement techniques are discussed. Details of a Portable RCS Measurement setup are covered.RCS measurement setup consists of a Handheld Vector Network Analyzer (VNA), Horn Antennas, and Pylon for supporting DUTs, stepper ...
Aniruddha Bose, Arijit De, Sankarsan Padhy; PXE,DRDO,Chandipur, R. Appavuraj   +3 more
core  

Peripheral artery disease in pseudoxanthoma elasticum: Walking capacity on the 6-minute walking test. [PDF]

Vasc Med
Haverkamp M, Visseren FL, Hazenberg CE, de Jong PA, Spiering W.   +4 more
europepmc   +1 more source