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Case Report: Nephrotic syndrome as the primary manifestation of Alport syndrome in a Chinese pediatric patient.
Front Pediatr
January 2025
Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu, China.
Background: Alport syndrome (AS) is a genetically heterogeneous disorder resulting from variants in genes coding for the alpha-3/4/5 chains of Collagen IV, leading to defective basement membranes in the kidney, cochlea, and eye. The clinical manifestations of AS vary in patients. Cases of childhood AS caused by presenting primarily with nephrotic syndrome (NS) are rarely reported.
View Article and Find Full Text PDFTargeting endoplasmic reticulum stress: an innovative therapeutic strategy for podocyte-related kidney diseases.
J Transl Med
January 2025
Endocrinology Department, First Affiliated Hospital, Changchun University of Chinese Medicine, Changchun, 130021, China.
The endoplasmic reticulum (ER) is a vital organelle responsible for protein quality control, including the folding, modification, and transport of proteins. When misfolded or unfolded proteins accumulate in the ER, it triggers endoplasmic reticulum stress (ERS) and activates the unfolded protein response (UPR) to restore ER homeostasis. However, prolonged or excessive ERS can lead to apoptosis.
View Article and Find Full Text PDFThe Link between Oxygen and Basement Membranes.
Rev Physiol Biochem Pharmacol
January 2025
Institute of Medical Sciences, University of Aberdeen, Aberdeen, Scotland, UK.
Epithelial tissues and the basement membranes they sit on did not appear for billions of years. Their appearance was delayed most likely by a lack of oxygen, which is required for collagen synthesis, and which only began to build up following the Great Oxygenation Event ~ 2.4 billion years ago.
View Article and Find Full Text PDFEpithelia Are Scaffolds for Electricity-Dependent Molecular Interactions.
Rev Physiol Biochem Pharmacol
January 2025
Institute of Medical Sciences, University of Aberdeen, Aberdeen, Scotland, UK.
Once multicellularity was thriving, a key development involved the emergence of epithelial layers that separated "inside" from "outside". Most epithelia then generate their own transepithelial electrical signals. So electrical forces were instrumental in the development of epithelial tissues, which themselves generate further electrical signals.
View Article and Find Full Text PDFβ1 Integrin/FAK signaling regulates interleukin-8 production in human gingival epithelial Ca9-22 cells.
J Oral Biosci
January 2025
Department of Physiology, Osaka Dental University, Osaka, Japan. Electronic address:
Objectives: Interleukin-8 (IL-8), a proinflammatory factor in human tissues, plays an important role in inflammation. Type IV collagen, a key component of the basement membrane, interacts with integrins, which are primary receptors in the extracellular matrix (ECM). Integrins are essential for the regulation of various cellular behaviors and signal transduction pathways.
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