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Mitochondrial HMG-CoA synthase deficiency.
Mol Genet Metab
January 2025
Clinical Department of Laboratory Medicine, University Hospitals Leuven, Leuven, Belgium; Department of Cardiovascular Sciences, KU Leuven, Leuven, Belgium. Electronic address:
Mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase 2 (HMGCS2) deficiency is a rare, potentially life-threatening autosomal recessive disorder resulting from mutations in the HMGCS2 gene, leading to impaired ketogenesis. We systematically reviewed the clinical presentations, biochemical and genetic abnormalities in 93 reported cases and 2 new patients diagnosed based on biochemical findings. Reported onset ages ranged from 3 months to 6 years, mostly before the age of 3.
View Article and Find Full Text PDFAutophagy related 7 (ATG7) regulates food intake and liver health during asparaginase exposure.
J Biol Chem
January 2025
Nutritional Sciences Graduate Program, Rutgers University, New Brunswick, New Jersey, United States; Rutgers Cancer Institute of New Jersey, New Brunswick, New Jersey, United States; Department of Nutritional Sciences, Rutgers University, New Brunswick, New Jersey, United States; Endocrinology and Animal Biosciences Graduate Program, Rutgers University, New Brunswick, New Jersey, United States; New Jersey Institute for Food, Nutrition and Health, Rutgers University, New Brunswick, New Jersey, United States. Electronic address:
Amino acid starvation by the chemotherapy agent asparaginase is a potent activator of the integrated stress response (ISR) in liver and can upregulate autophagy in some cell types. We hypothesized that autophagy related 7 (ATG7), a protein that is essential for autophagy and an ISR target gene, was necessary during exposure to asparaginase to maintain liver health. We knocked down Atg7 systemically (Atg7) or in hepatocytes only (ls-Atg7KO) in mice before exposure to pegylated asparaginase for 5 d.
View Article and Find Full Text PDFMeat intake in relation to composition and function of gut microbiota.
Clin Nutr
January 2025
Molecular Epidemiology, Department of Medical Sciences, Uppsala University, Uppsala, Sweden; Preventive Medicine Division, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, United States; School of Natural Sciences, Technology and Environmental Studies, Södertörn University Sweden, Sweden. Electronic address:
Objective: Meat intake is suggested to affect gut microbiome composition and the risk of chronic diseases. We aimed to identify meat-associated gut microbiome features and their association with host factors.
Design: Gut microbiota species were profiled by deep shotgun metagenomics sequencing in 9669 individuals.
The relationship between dietary branched-chain and aromatic amino acids with the regulation of leptin and FTO genes in adipose tissue of patients undergoing abdominal surgery.
Amino Acids
January 2025
Department of Nutrition, School of Public Health, Iran University of Medical Sciences, Tehran, Iran.
Recent studies have suggested that the interaction between diet and an individual's genetic predisposition can determine the likelihood of obesity and various metabolic disorders. The current study aimed to examine the association of dietary branched-chain amino acids(BCAAs) and aromatic amino acids(AAAs) with the expression of the leptin and FTO genes in the visceral and subcutaneous adipose tissues of individuals undergoing surgery. This cross-sectional study was conducted on 136 Iranian adults, both men and women, aged ≥18 years.
View Article and Find Full Text PDFCan We Maintain Muscle Mass on a Plant-Based Diet?
Curr Nutr Rep
January 2025
Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA.
Purpose Of Review: This review aims to determine whether muscle mass and function can be effectively maintained without relying on animal-based protein sources. We evaluate the quality, digestibility, and essential amino acid profiles of plant-based proteins to understand their potential in preventing and managing sarcopenia.
Recent Finding: Recent studies indicate that while animal-based proteins have traditionally been considered the gold standard for supporting muscle protein synthesis, certain plant-based protein blends, fortified with leucine or other essential amino acids, can produce comparable anabolic responses.
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