A patient with very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency presented in the neonatal period with hypoketotic hypoglycaemia and at the age of 1 year with rhabdomyolysis and normal glucose after fasting. Rhabdomyolysis may occur in the absence of hypoglycaemia in young infants as well as in older patients.
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http://dx.doi.org/10.1007/s10545-005-0190-y | DOI Listing |
Nutrients
January 2025
Department of Food & Nutrition, Kyung Hee University, Seoul 02447, Republic of Korea.
Background/objectives: The pathogenesis of metabolic dysfunction-associated steatohepatitis (MASH) is closely associated with increased oxidative stress and lipid peroxidation. Coenzyme Q (CoQ) and selenium (Se) are well-established antioxidants with protective effects against oxidative damage. This study aimed to investigate the effects of CoQ and Se in ameliorating MASH induced by a methionine choline-deficient (MCD) diet in mice.
View Article and Find Full Text PDFAnn Clin Lab Sci
November 2024
Emergency Department, Wenzhou Third Clinical Institute Affiliated to Wenzhou Medical University, Wenzhou People's Hospital, Wenzhou, Zhejiang, China
Objective: Myocardial injury is a prevalent complication of sepsis. This study aims to shed light on the role of Acyl-CoA Synthetase Long Chain Family Member 4 (ACSL4) in regulating Fatty Acid Synthase (FASN) to identify the intrinsic molecular mechanisms of sepsis-induced myocardial injury.
Method: H9c2 cells were treated with Lipopolysaccharide (LPS) to model sepsis-induced cardiomyocyte injury and were subsequently divided into seven groups: Control, LPS, LPS+sh-NC, LPS+sh-ACSL4, LPS+sh-ACSL4+Erastin, LPS+sh-ACSL4+oe-NC, and LPS+sh-ACSL4+oe-FASN.
Sheng Wu Gong Cheng Xue Bao
January 2025
College of Biotechnology and Bioengineering, Zhejiang University of Technology, Hangzhou 310014, Zhejiang, China.
O-acetyl-L-homoserine (OAH) is a promising platform compound for the production of L-methionine and other valuable compounds, while its low yield and low conversion rate limit the industrial application. To solve these problems, we constructed a strain for high OAH production with the previously constructed L-homoserine producer HS33 as the chassis by systematic metabolic engineering. Firstly, PEP accumulation, pyruvate utilization, and OAH synthesis pathway (overexpressing , , and ) were enhanced to obtain an initial strain accumulating 13.
View Article and Find Full Text PDFAdv Sci (Weinh)
January 2025
Shanghai Key Laboratory of Vascular Lesions and Remodeling, Department of Vascular Surgery, Shanghai Pudong Hospital, Fudan University Pudong Medical Center, Shanghai, 201399, China.
Acute myocardial infarction (AMI) is associated with well-established metabolic risk factors, especially hyperlipidemia and obesity. Myocardial ischemia-reperfusion injury (mIRI) significantly offsets the therapeutic efficacy of revascularization. Previous studies indicated that disrupted lipid homeostasis can lead to lipid peroxidation damage and inflammation, yet the underlying mechanisms remain unclear.
View Article and Find Full Text PDFMol Oncol
January 2025
Center for Molecular Medicine, MaineHealth Institute for Research, Scarborough, ME, USA.
Multiple myeloma (MM) is an incurable cancer of plasma cells with a 5-year survival rate of 59%. Dysregulation of fatty acid (FA) metabolism is associated with MM development and progression; however, the underlying mechanisms remain unclear. Herein, we explore the roles of long-chain fatty acid coenzyme A ligase (ACSL) family members in MM.
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