Results 51 to 60 of about 5,571 (225)
Impact of N‐linked glycans on the dual short fibulin/LTBP‐4 axes regulating elastogenesis
The FEBS Journal, EarlyView.Elastic fiber assembly is orchestrated by multiple glycoproteins. We delineate the molecular basis of two key axes involved in elastic fiber formation—LTBP‐4L/fibulin‐4 and LTBP‐4S/fibulin‐5. We show that N‐linked glycans on these glycoproteins regulate their interactions and conformations, both of which are critical aspects in elastic fiber formation.
Valentin Nelea +8 more
wiley +1 more source
Novel deletions causing pseudoxanthoma elasticum underscore the genomic instability of the ABCC6 region [PDF]
, 2010 Mutations in ABCC6 cause pseudoxanthoma elasticum (PXE), a heritable disease that affects elastic fibers. Thus far, >200 mutations have been characterized by various PCR-based techniques (primarily direct sequencing), identifying up to 90% of PXE-causing
Chassaing, N +9 more
core +2 more sources
Magnetic Resonance in Medicine, Volume 94, Issue 2, Page 771-784, August 2025.Abstract Purpose The aim is to establish the relationship between carotid susceptibility and microstructural components in diseased carotid arteries. Methods Excised cadaveric carotid arteries (n = 5) were scanned using high‐resolution QSM at 7 Tesla. After ex vivo imaging, all samples were brought to histology and stained for elastin, collagen, cells,
Alan J. Stone +4 more
wiley +1 more source
Primary systemic amyloidosis, acquired cutis laxa and cutaneous mucinosis in a patient with multiple myeloma [PDF]
Anais Brasileiros de Dermatologia, 2013 A 57-year-old woman presented with periorbital ecchymoses, laxity in skin folds, polyneuropathy and bilateral carpal tunnel syndrome. A skin biopsy of the axillary lesion demonstrated fragmentation of elastic fibers, but with a negative von Kossa stain ...
Fernanda Guedes Lavorato +5 more
doaj +1 more source
Clinical Case Reports, Volume 13, Issue 7, July 2025.ABSTRACT This case highlights the importance of considering rare conditions like Mounier‐Kuhn Syndrome in patients with chronic respiratory symptoms, especially in regions with high tuberculosis prevalence. Enhanced awareness and advanced imaging are vital for accurate diagnosis and effective management of such conditions.
Girma Deshimo, Enguday Demeke
wiley +1 more source
Clinical Features and Brain MRI Findings in Korean Patients with AGel Amyloidosis [PDF]
, 2021 Purpose: AGel amyloidosis is systemic amyloidosis caused by pathogenic variants in the GSN gene. In this study, we sought to characterize the clinical and brain magnetic resonance image (MRI) features of Korean patients with AGel amyloidosis.
박형준 +3 more
core +1 more source
Journal of Cosmetic Dermatology, Volume 24, Issue 7, July 2025.ABSTRACT Purpose This study aims to explore the mechanism of Bazi Bushen Capsule (BZBS) in treating skin laxity by combining network pharmacology and clinical research. Methods The active ingredients and potential drug targets of BZBS were obtained from TCMSP, TCMBANK, and SuperTCM databases.
Mo Zhao +12 more
wiley +1 more source
Autosomal recessive cutis laxa type-1 with complex systemic manifestations
Indian Journal of Paediatric Dermatology, 2018 Cutis laxa (CL) is a rare connective tissue disorder characterized by loose, sagging, and redundant skin. Both inherited and acquired forms are known. A 7-year-old boy, presented with loose, sagging skin chiefly over the face, extremities, and skin folds
Shruti Dhanraj Chavan +4 more
doaj +1 more source
Cutis laxa with pulmonary artery stenosis
Indian Dermatology Online Journal, 2012 Krina B Patel, Ruchin Patel
doaj +4 more sources
Journal of Experimental and Clinical Medicine, 2011 ABSTRACT Cutis laxa is an uncommon disorder characterized clinically laxitiy of the skin, which hangs in loose folds, by the loss of dermal elastic tissue. We report a case only neck localized of cutis laxa that no systemic involvement was diagnosed. The skin laxity was preceded by episodes of itching and swelling on her neck. When the patient applied
TUNCEL, Umut +3 more
openaire +5 more sources