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Serum fatty acid profiling in patients with SDHx mutations: New advances on cellular metabolism in SDH deficiency.
Biochimie
October 2022
CHU Lille, Laboratoire de Biochimie, Hormonologie-Métabolisme-Nutrition-Oncologie, F-59037, Lille, France. Electronic address:
Apart from the oncometabolite succinate, little studies have appeared on extra-mitochondrial pathways in Succinate Dehydrogenase (SDH) genetic deficiency. The role of NADH/NAD redox status and dependent pathways was recently emphasized. Therein, fatty acid (FA) metabolism data were collected here in 30 patients with a loss of function (LOF) variant in one SDHx gene (either with a pheochromocytoma/paraganglioma (PPGL) or asymptomatic) and in 22 wild-type SDHx controls (with PPGL or asymptomatic).
View Article and Find Full Text PDFMice with a deficiency in Peroxisomal Membrane Protein 4 (PXMP4) display mild changes in hepatic lipid metabolism.
Sci Rep
February 2022
Department of Pediatrics, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
Peroxisomes play an important role in the metabolism of a variety of biomolecules, including lipids and bile acids. Peroxisomal Membrane Protein 4 (PXMP4) is a ubiquitously expressed peroxisomal membrane protein that is transcriptionally regulated by peroxisome proliferator-activated receptor α (PPARα), but its function is still unknown. To investigate the physiological function of PXMP4, we generated a Pxmp4 knockout (Pxmp4) mouse model using CRISPR/Cas9-mediated gene editing.
View Article and Find Full Text PDFphytanoyl-CoA dioxygenase domain-containing protein 1 plays an important role in egg shell formation of silkworm (Bombyx mori).
PLoS One
January 2022
The Sericultural and Apicultural Research Institute, Yunnan Academy of Agricultural Sciences, Mengzi, Yunnan, China.
Yun7Ge is a giant egg mutant found in the silkworm variety Yun7. In comparison with the giant mutant Ge, the eggs of Yun7Ge are larger. The number of laid eggs and hatching rate of Yun7Ge are reduced, which is not conducive to reproduction.
View Article and Find Full Text PDFBranched-Chain Fatty Acids-An Underexplored Class of Dairy-Derived Fatty Acids.
Nutrients
September 2020
Department of Animal and Veterinary Sciences, The University of Vermont, Burlington, VT 05405, USA.
Dairy fat and its fatty acids (FAs) have been shown to possess pro-health properties that can support health maintenance and disease prevention. In particular, branched-chain FAs (BCFAs), comprising approximately 2% of dairy fat, have recently been proposed as bioactive molecules contributing to the positive health effects associated with the consumption of full-fat dairy products. This narrative review evaluates human trials assessing the relationship between BCFAs and metabolic risk factors, while potential underlying biological mechanisms of BCFAs are explored through discussion of studies in animals and cell lines.
View Article and Find Full Text PDFPhytanic acid-derived peroxisomal lipid metabolism in porcine oocytes.
Theriogenology
November 2020
Department of Theriogenology and Biotechnology, Research Institute for Veterinary Science, College of Veterinary Medicine, Seoul National University, Seoul, Republic of Korea. Electronic address:
Lipid metabolism plays an important role in oocyte maturation. The peroxisome is the fundamental mediator for this mechanism. In this study, we investigated the peroxisomal lipid metabolism in porcine oocytes.
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