Results 11 to 20 of about 2,564 (158)

Relationship Between Mitochondrial Structure and Bioenergetics in Pseudoxanthoma elasticum Dermal Fibroblasts [PDF]

Frontiers in Cell and Developmental Biology, 2020
Pseudoxanthoma elasticum (PXE) is a genetic disease considered as a paradigm of ectopic mineralization disorders, being characterized by multisystem clinical manifestations due to progressive calcification of skin, eyes, and the cardiovascular system ...
Francesco Demetrio Lofaro, Federica Boraldi, Maria Garcia-Fernandez, Lara Estrella, Pedro Valdivielso, Pedro Valdivielso, Daniela Quaglino   +6 more
doaj   +4 more sources

Automated system for deploying operating systems using LAN (PXE SERVER) [PDF]

Литьë и металлургия, 2022
PXE is an environment for booting computers using a network card without using hard drives, CDs and other devices used while booting operating systems.
I. V. Kendysh
doaj   +3 more sources

Plasma lipids in Pseudoxanthoma Elasticum (PXE) patients: A comparative study with population-based reference values and Non-PXE controls [PDF]

Atherosclerosis Plus
Background and aims: – Pseudoxanthoma elasticum (PXE) is a rare genetic disease caused by pathogenic mutations in the ABCC6 gene, resulting in low values of inorganic pyrophosphate (PPi).
Iris M. Harmsen, Frank L.J. Visseren, Madeleine Kok, Pim A. de Jong, Wilko Spiering   +4 more
doaj   +3 more sources

The Activation of JAK/STAT3 Signaling and the Complement System Modulate Inflammation in the Primary Human Dermal Fibroblasts of PXE Patients

Biomedicines, 2023
Previous studies revealed a link between inflammation and overactivation of the Janus kinase (JAK)/signal transducer and activator of transcription (STAT) signaling in syndromes associated with aging.
Christopher Lindenkamp, Ricarda Plümers, Michel R. Osterhage, Olivier M. Vanakker, Judith Van Wynsberghe, Cornelius Knabbe, Doris Hendig   +6 more
doaj   +4 more sources

Various vascular malformations are prevalent in Finnish pseudoxanthoma elasticum (PXE) patients: a national registry study

Orphanet Journal of Rare Diseases, 2022
Background Pseudoxanthoma elasticum (PXE, OMIM# 264800) is an inborn error of metabolism causing ectopic soft tissue calcification due to low plasma pyrophosphate concentration.
Saku Pelttari, Suvi Väärämäki, Olivier Vanakker, Shana Verschuere, Hannu Uusitalo, Heini Huhtala, Tero Hinkka, Ilkka Pörsti, Pasi I. Nevalainen   +8 more
doaj   +2 more sources

Inorganic pyrophosphate plasma levels in patients with GGCX-associated PXE-like phenotypes [PDF]

Frontiers in Genetics
IntroductionPseudoxanthoma elasticum (PXE) is an autosomal recessive ectopic calcification disorder clinically affecting the skin, eyes, and vascular system.
Qiaoli Li, Qiaoli Li, Catherine Troutman, Mary Peckiconis, Tamara Wurst, Sharon F. Terry   +5 more
doaj   +2 more sources

Nonfamilial Pseudoxanthoma Elasticum: A Case Report With Review of Selected Literature. [PDF]

Clin Case Rep
ABSTRACT A 44‐year‐old woman with classic cutaneous manifestations of nonfamilial PXE presented with multiple 3 to 10 mm yellowish papules coalescing into well‐demarcated, cobblestone‐like plaques. Previous ophthalmologic evaluation revealed retinal angioid streaks, while no cardiovascular involvement was reported.
Peniche-Luna E, Uriarte-Ruiz K, Pérez-Nieto JE, Briseño-Gascón G, Vega-Memije ME, Guzmán-Bucio S.   +5 more
europepmc   +2 more sources

Severe Hypertension and Hematologic Abnormalities in Pseudoxanthoma Elasticum: A Pediatric Case Report. [PDF]

J Clin Hypertens (Greenwich)
ABSTRACT Pseudoxanthoma elasticum (PXE) is a rare autosomal recessive disorder caused by pathogenic variants in ABCC6, leading to progressive calcification of elastic fibers. Although PXE typically presents in adolescence with dermatologic or ocular manifestations, early vascular involvement, including pediatric hypertension, is increasingly recognized.
Flogelova H, Pospisilova D, Bakaj-Zbrozkova L, Sochorcova L, Manakova J, Kriegova E, Koralkova P, Hlusickova Kapralova K, Horvathova M.   +8 more
europepmc   +2 more sources

TPCM-based trusted PXE boot method for servers

网络与信息安全学报, 2020
The PXE startup mechanism downloads operating system files through the network and starts the operating system,which is widely used in server network startup.It is widely used in server network startup.The PXE boot process is secured and trusted through ...
Guojie LIU, Jianbiao ZHANG
doaj   +3 more sources

Skin lesions over the neck with vision loss: Pseudoxanthoma elasticum with angioid streaks

TNOA Journal of Ophthalmic Science and Research, 2020
Pseudoxanthoma elasticum (PXE) is an autosomal recessive multisystem disorder characterized by the involvement of skin, cardiovascular system, and the eyes. Angioid streaks (AS) are the most common ophthalmoscopic finding in PXE.
Gauri Khare, Chitaranjan Mishra, Naresh Babu Kannan   +2 more
doaj   +1 more source