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Gastrointestinal complications of hepatic glycogen storage disease: a national survey questionnaire study in China.
Orphanet J Rare Dis
January 2025
Department of Pediatrics, Guangdong Provincial People's Hospital, The Second School of Clinical Medicine, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, 510080, China.
Background: Hepatic glycogen storage diseases (GSD) are inborn errors of metabolism with abnormal storage or utilization of glycogen, a complex disease with significant genetic heterogeneity and similar clinical manifestations. This study aimed to describe the gastrointestinal symptoms and endoscopic features of hepatic GSD, including types Ia, Ib, III, VI, and IX, to provide evidence for etiology and treatment.
Methods: A national cohort survey questionnaire was distributed to patients diagnosed with GSD type Ia, Ib, III, VI, and IX through genetic testing or their parents in mainland China in May 2022.
Exploring the Internal Environmental Changes of Muscle Cells and Apoptotic Phase of Mitochondria in Dry-Cured Loin Using Electrical Stimulation: Promoting the Precise Regulation of Loin Ham Quality.
J Agric Food Chem
January 2025
Guizhou Key Laboratory of New Quality Processing and Storage of Ecological Specialty Food; School of Liquor and Food Engineering, Guizhou University, Guiyang 550025, China.
Traditional dry-curing methods have a long cycle time and low efficiency, resulting in the inconsistent quality of dry-cured ham. By applying electrical stimulation (ES) technology in the dry-curing process, it was found that ES affected mitochondrial apoptosis by modulating the intracellular environment of muscle cells, which, in turn, enhanced the quality of dry-cured pork loin. Specifically, ES accelerated glycogen and ATP depletion, which led to a rapid decline in pH.
View Article and Find Full Text PDFUnderstanding the electron pathway fluidity of Synechocystis in biophotovoltaics.
Plant J
January 2025
Systems Biotechnology Group, Department Microbial Biotechnology, Helmholtz Centre for Environmental Research - UFZ, Leipzig, 04318, Germany.
Biophotovoltaics offers a promising low-carbon footprint approach to utilize solar energy. It aims to couple natural oxygenic photosynthetic electrons to an external electron sink. This lays the foundation for a potentially high light-to-energy efficiency of the Biophotovoltaic process.
View Article and Find Full Text PDFGlycogen storage disease type III (GSD III) is a rare metabolic disorder characterized by a deficiency of liver and muscle amylo-1,6-glucosidase. This condition presents with severe hepatic symptoms in childhood, mostly hepatomegaly, hypoglycemia in half of patients, while muscular complications may predominate in adulthood. Hepatic fibrosis, cirrhosis and hepatocellular carcinoma (HCC) are common complications in older patients.
View Article and Find Full Text PDFVEXAS, Chediak-Higashi syndrome and Danon disease: myeloid cell endo-lysosomal pathway dysfunction as a common denominator?
Cell Mol Biol Lett
January 2025
University Cote d'Azur, Inserm, C3M, Nice, France.
Vacuolization of hematopoietic precursors cells is a common future of several otherwise non-related clinical settings such as VEXAS, Chediak-Higashi syndrome and Danon disease. Although these disorders have a priori nothing to do with one other from a clinical point of view, all share abnormal vacuolization in different cell types including cells of the erythroid/myeloid lineage that is likely the consequence of moderate to drastic dysfunctions in the ubiquitin proteasome system and/or the endo-lysosomal pathway. Indeed, the genes affected in these three diseases UBA1, LYST or LAMP2 are known to be direct or indirect regulators of lysosome trafficking and function and/or of different modes of autophagy.
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