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Maple syrup has nutraceutical potential given the macronutrients (carbohydrates, primarily sucrose), micronutrients (minerals and vitamins), and phytochemicals (primarily phenolics) found in this natural sweetener. We conducted compositional (ash, fiber, carbohydrates, minerals, amino acids, organic acids, vitamins, phytochemicals), in vitro biological, and in vivo safety (animal toxicity) studies on maple syrup extracts (MSX-1 and MSX-2) derived from two declassified maple syrup samples. Along with macronutrient and micronutrient quantification, thirty-three phytochemicals were identified (by HPLC-DAD), and nine phytochemicals, including two new compounds, were isolated and identified (by NMR) from MSX. At doses of up to 1000 mg/kg/day, MSX was well tolerated with no signs of overt toxicity in rats. MSX showed antioxidant (2,2-diphenyl-1-picrylhydrazyl (DPPH) assay) and anti-inflammatory (in RAW 264.7 macrophages) effects and inhibited glucose consumption (by HepG2 cells) in vitro. Thus, MSX should be further investigated for potential nutraceutical applications given its similarity in chemical composition to pure maple syrup.
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http://dx.doi.org/10.1021/jf501924y | DOI Listing |
Neurochem Res
September 2025
Laboratório de Doenças Neurometabólicas, Programa de Pós-Graduação Em Ciências da Saúde, Universidade Do Extremo Sul Catarinense, Criciúma, SC, 88806-000, Brazil.
Branched-chain amino acids (BCAA) leucine, isoleucine, and valine are metabolized by complex branched-chain ketoacids dehydrogenase (BCKDH). In Maple Syrup Urine Disease (MSUD), the BCKDH complex has its activity blocked by a genetic mutation, compromising the BCAA metabolism and leading to the accumulation of these BCAA, related to neurological damage in this disease. Thus, minocycline is a broad-spectrum antibiotic, bacteriostatic, and studies have shown benefits in neurodegenerative disease progression, like reduction of oxidative stress, inflammation, and downregulation of molecular pathways, such as apoptosis.
View Article and Find Full Text PDFJ Clin Res Pediatr Endocrinol
August 2025
Department of Pediatric Metabolism, Ankara University Faculty of Medicine, Ankara, Turkiye.
Maple Syrup Urine Disease (MSUD) and Type 1 Diabetes Mellitus (T1DM) are two distinct metabolic disorders with unique dietary management requirements. While MSUD necessitates strict restriction of branched-chain amino acids (BCAAs), T1DM requires precise carbohydrate counting to maintain optimal glycemic control. We report two cases of patients diagnosed with both MSUD and T1DM, highlighting the challenges and strategies in dietary management.
View Article and Find Full Text PDFMol Genet Metab Rep
September 2025
Hayward Genetics Center, Tulane University School of Medicine, New Orleans, LA, United States of America.
Maple syrup urine disease (MSUD) is a rare autosomal recessive metabolic disorder caused by a deficiency of the branched-chain α-ketoacid dehydrogenase (BCKAD) complex. It is classified into four subtypes: classic, intermediate, intermittent, and thiamine-responsive. We report a case of a female infant who presented with global developmental delay at 8 months of age.
View Article and Find Full Text PDFAm J Clin Nutr
August 2025
Institut d'Investigació Sanitària Pere Virgil i (IISPV), Grup Alimentació, Nutrició, Desenvolupament i Salut Mental, Reus, Spain; Universitat Rovira i Virgili, Departament de Bioquímica i Biotecnologia, Alimentació, Nutrició, Desenvolupament i Salut Mental ANUT-DSM, Reus, Spain; Centro de Inv
Commun Med (Lond)
July 2025
Department of Biochemistry and Molecular Genetics, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Background: Diabetic ketoacidosis is an acute, potentially life-threatening, metabolic complication and often first presentation of type 1 diabetes (T1D) mellitus. Here, we investigated the metabolic and lipid profiles from pediatric patients with T1D, at initial diagnosis and after two weeks of insulin treatment, employing findings from patients affected by maple syrup urine disease (MSUD) and the Recipient Epidemiology and Donor Evaluation Study (REDS) III RBC Omics.
Methods: 27 patients with newly onset T1D were assessed at the University of Campania "L.