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Brain Branched-Chain Amino Acids in Maple Syrup Urine Disease: Implications for Neurological Disorders

https://doi.org/10.3390/ijms21207490

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What points at this work

Incoming claims: where this work appears in other papers’ reference lists and arguments.

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inferred← citeshttps://doi.org/10.1002/ajmg.a.63129openalex-snapshot

PPM1K defects cause mild maple syrup urine disease: The second case in the literature

inferred← citeshttps://doi.org/10.1002/jdn.10283openalex-snapshot

Treatment of maple syrup urine disease: Benefits, risks, and challenges of liver transplantation

inferred← citeshttps://doi.org/10.1002/jmd2.12419openalex-snapshot

Computational structural genomics and clinical evidence suggest BCKDK gain‐of‐function may cause a potentially asymptomatic maple syrup urine disease phenotype

inferred← citeshttps://doi.org/10.1007/978-3-030-94510-7_17openalex-snapshot

Nutrition Management of Maple Syrup Urine Disease

inferred← citeshttps://doi.org/10.1007/978-3-031-58843-3_13openalex-snapshot

Mitochondrial Alpha-Keto Acid Dehydrogenase Complexes: Recent Developments on Structure and Function in Health and Disease

inferred← citeshttps://doi.org/10.1007/s00406-025-02147-0openalex-snapshot

Genetic evidence on chemical communication between gut microbiota and neurological and psychiatric disorders: a Mendelian randomization study

inferred← citeshttps://doi.org/10.1007/s00726-024-03417-2openalex-snapshot

Branched-chain amino acids: physico-chemical properties, industrial synthesis and role in signaling, metabolism and energy production

inferred← citeshttps://doi.org/10.1007/s10522-025-10309-9openalex-snapshot

Role of branched chain amino acid metabolism on aging

inferred← citeshttps://doi.org/10.1007/s11011-022-00969-8openalex-snapshot

Coadministration of tianeptine alters behavioral parameters and levels of neurotrophins in a chronic model of Maple Syrup Urine disease

inferred← citeshttps://doi.org/10.1007/s11011-022-01070-wopenalex-snapshot

Exposure to leucine alters glutamate levels and leads to memory and social impairment in zebrafish

inferred← citeshttps://doi.org/10.1007/s11064-025-04549-xopenalex-snapshot

Minocycline Protects Against Oxidative Stress in a Model of Maple Syrup Urine Disease

inferred← citeshttps://doi.org/10.1007/s11270-024-07646-9openalex-snapshot

Combating Lead (Pb) Contamination: Integrating Biomonitoring, Advanced Detection, and Remediation for Environmental and Public Health

inferred← citeshttps://doi.org/10.1007/s12031-026-02476-2openalex-snapshot

Neuronal Metabolism of Branched-Chain Amino Acids Alleviates Lifespan Reduction Caused by High-Salt Diet in Drosophila

inferred← citeshttps://doi.org/10.1007/s12265-024-10479-wopenalex-snapshot

The Role of Branched-chain Amino Acids and Their Metabolism in Cardiovascular Diseases

inferred← citeshttps://doi.org/10.1007/s12672-023-00634-1openalex-snapshot

High dose isoleucine stabilizes nuclear PTEN to suppress the proliferation of lung cancer

inferred← citeshttps://doi.org/10.1016/b978-0-12-824412-8.00006-0openalex-snapshot

Nutrigenomics in the management and prevention of metabolic disorders

inferred← citeshttps://doi.org/10.1016/b978-0-443-10944-7.00097-4openalex-snapshot

Inherited Metabolic Epilepsies

inferred← citeshttps://doi.org/10.1016/b978-0-443-36312-2.00006-1openalex-snapshot

Amino acid and protein metabolism disorders

inferred← citeshttps://doi.org/10.1016/b978-0-443-36312-2.00016-4openalex-snapshot

Organic acid metabolism disorders

inferred← citeshttps://doi.org/10.1016/b978-0-443-44534-7.00016-8openalex-snapshot

Metabolism of individual amino acids in health and disease

inferred← citeshttps://doi.org/10.1016/j.anrea.2022.11.003openalex-snapshot

Sirop d’érable et sauce soja

inferred← citeshttps://doi.org/10.1016/j.bpsgos.2024.100339openalex-snapshot

Unraveling the Molecular Mechanisms of the Neurodevelopmental Consequences of Fetal Protein Deficiency: Insights From Rodent Models and Public Health Implications

inferred← citeshttps://doi.org/10.1016/j.cbpc.2026.110457openalex-snapshot

Metabolomic insights into gut–brain axis dysregulation under unpredictable chronic stress in zebrafish

inferred← citeshttps://doi.org/10.1016/j.cca.2023.117483openalex-snapshot

Newborn screening of maple syrup urine disease and the effect of early diagnosis

inferred← citeshttps://doi.org/10.1016/j.heliyon.2026.e44658openalex-snapshot

Exploring the intersecting paths between metabolic disorders and neurological manifestations

inferred← citeshttps://doi.org/10.1016/j.jad.2025.03.032openalex-snapshot

Association of branched-chain amino acids with major depressive disorder: A bidirectional Mendelian randomization study

inferred← citeshttps://doi.org/10.1016/j.jep.2022.116038openalex-snapshot

Gut microbiota: A new target for traditional Chinese medicine in the treatment of depression

inferred← citeshttps://doi.org/10.1016/j.jpeds.2021.06.028openalex-snapshot

Metabolic Control and “Ideal” Outcomes in Liver Transplantation for Maple Syrup Urine Disease

inferred← citeshttps://doi.org/10.1016/j.jpsychores.2025.112101openalex-snapshot

Circulating branched-chain amino acids and risk of psychiatric disorders: A two-sample Mendelian randomization study

inferred← citeshttps://doi.org/10.1016/j.neubiorev.2021.11.012openalex-snapshot

ADHD symptoms in neurometabolic diseases: Underlying mechanisms and clinical implications

inferred← citeshttps://doi.org/10.1016/j.nut.2022.111762openalex-snapshot

Potential roles of branched-chain amino acids in neurodegeneration

inferred← citeshttps://doi.org/10.1016/j.talanta.2023.124465openalex-snapshot

Determination of creatinine in urine and blood serum human samples by CZE-UV using on-column internal standard injection

inferred← citeshttps://doi.org/10.1016/j.xhgg.2025.100525openalex-snapshot

Bi-allelic variants in BCAT1 impair mitochondrial function and are associated with a candidate neurometabolic disorder

inferred← citeshttps://doi.org/10.1016/j.ymgmr.2024.101091openalex-snapshot

Partial suppression of BCAA catabolism as a potential therapy for BCKDK deficiency

inferred← citeshttps://doi.org/10.1038/s41531-025-01255-xopenalex-snapshot

Metabolomic breath landscape analysis unravels lipid biomarker candidates in patients with genetic and idiopathic Parkinson’s disease

inferred← citeshttps://doi.org/10.1038/s41598-021-02591-7openalex-snapshot

Branched-chain amino acids govern the high learning ability phenotype in Tokai high avoider (THA) rats

inferred← citeshttps://doi.org/10.1038/s41598-024-70275-zopenalex-snapshot

Choline chloride and amino acid solutions taste and hydration behavior with experimental thermodynamic properties and COSMO-PC-SAFT calculation

inferred← citeshttps://doi.org/10.1038/s42003-025-07457-6openalex-snapshot

Dysfunctional BCAA degradation triggers neuronal damage through disrupted AMPK-mitochondrial axis due to enhanced PP2Ac interaction

inferred← citeshttps://doi.org/10.1080/19490976.2026.2698204openalex-snapshot

Branched-chain amino acids and gut microbiota: coregulation and impact on neurological function via the gut-brain axis

inferred← citeshttps://doi.org/10.1093/cercor/bhaf040openalex-snapshot

Functional connectivity changes in mouse models of maple syrup urine disease

inferred← citeshttps://doi.org/10.1098/rsob.250092openalex-snapshot

Brain amino acid sensing for organismal amino acid homeostasis

inferred← citeshttps://doi.org/10.1101/2022.06.10.495711openalex-snapshot

Nervous system reduction in branched-chain amino acid metabolism disrupts hippocampal neurogenesis and memory

inferred← citeshttps://doi.org/10.1101/2023.12.18.572239openalex-snapshot

Branched-chain keto acids promote an immune-suppressive and neurodegenerative microenvironment in leptomeningeal disease

inferred← citeshttps://doi.org/10.1101/2025.04.17.25326025openalex-snapshot

Metabolomic breath landscape analysis unravels lipid biomarker candidates in patients with monogenic and idiopathic Parkinson’s disease

inferred← citeshttps://doi.org/10.1111/ped.70353openalex-snapshot

A case of maple syrup urine disease with infantile epileptic spasms syndrome: Challenges in a resource‐limited setting

inferred← citeshttps://doi.org/10.1124/pharmrev.123.000886openalex-snapshot

The SLC6A15–SLC6A20 Neutral Amino Acid Transporter Subfamily: Functions, Diseases, and Their Therapeutic Relevance

inferred← citeshttps://doi.org/10.1148/rg.240053openalex-snapshot

Congenital Inborn Errors of Metabolism: Clinical and Imaging Pearls

inferred← citeshttps://doi.org/10.1186/s12887-024-05266-0openalex-snapshot

Maple syrup urine disease diagnosed in a resource-limited setting in an infant in Nepal: a case report

inferred← citeshttps://doi.org/10.1186/s13023-022-02353-2openalex-snapshot

Intravenous administration of a branched-chain amino-acid-free solution in children and adults with acute decompensation of maple syrup urine disease: a prospective multicentre observational study

inferred← citeshttps://doi.org/10.1186/s13023-025-04043-1openalex-snapshot

Cerebral edema in maple syrup urine disease: spectrum of clinical presentation and treatment outcomes

inferred← citeshttps://doi.org/10.1186/s13023-026-04418-yopenalex-snapshot

Management of acute metabolic decompensation in maple syrup urine disease: guidance based on international clinical practice

inferred← citeshttps://doi.org/10.1364/translational.2022.jm3a.56openalex-snapshot

Resting-State Optical Neuroimaging in a Mouse Model of Metabolic Crisis in Maple Syrup Urine Disease

inferred← citeshttps://doi.org/10.1371/journal.pgen.1011407openalex-snapshot

Genetic risk for neurodegenerative conditions is linked to disease-specific microglial pathways

inferred← citeshttps://doi.org/10.14336/ad.2024.0538openalex-snapshot

Gut Microbiota and Its Metabolites: The Emerging Bridge Between Coronary Artery Disease and Anxiety and Depression?

inferred← citeshttps://doi.org/10.15252/embr.202356958openalex-snapshot

Defective branched‐chain amino acid catabolism in dorsal root ganglia contributes to mechanical pain

inferred← citeshttps://doi.org/10.21203/rs.3.rs-10261945/v1openalex-snapshot

ICAM-1, BDNF, and Neurobehavioral Profiles in Maple Syrup Urine Disease: Exploring Potential Biomarker Links

inferred← citeshttps://doi.org/10.3233/jad-221147openalex-snapshot

Insulin Resistance and Impaired Branched-Chain Amino Acid Metabolism in Alzheimer’s Disease

inferred← citeshttps://doi.org/10.3389/fcell.2020.603837openalex-snapshot

Metabolism of Amino Acids in Cancer

inferred← citeshttps://doi.org/10.3389/fcell.2021.762500openalex-snapshot

Metabolite Changes in the Aqueous Humor of Patients With Retinal Vein Occlusion Macular Edema: A Metabolomics Analysis

inferred← citeshttps://doi.org/10.3389/fnagi.2022.866886openalex-snapshot

The Big Picture of Neurodegeneration: A Meta Study to Extract the Essential Evidence on Neurodegenerative Diseases in a Network-Based Approach

inferred← citeshttps://doi.org/10.3389/fped.2022.933081openalex-snapshot

Case report: NAFLD and maple syrup urine disease: Is there an interplay between branched-chain amino acids and fructose consumption?

inferred← citeshttps://doi.org/10.3389/fped.2022.969741openalex-snapshot

Intravenous branched-chain amino-acid-free solution for the treatment of metabolic decompensation episodes in Spanish pediatric patients with maple syrup urine disease

inferred← citeshttps://doi.org/10.3390/biology11091308openalex-snapshot

Current Understanding on the Genetic Basis of Key Metabolic Disorders: A Review

inferred← citeshttps://doi.org/10.3390/diseases13030076openalex-snapshot

Setting the Stage for Branched-Chain Amino Acids Use in Neurological Pathologies: Does a Single Oral Dose Provide Hours of Elevated Systemic Levels?

inferred← citeshttps://doi.org/10.3390/ijms23074022openalex-snapshot

The Critical Role of the Branched Chain Amino Acids (BCAAs) Catabolism-Regulating Enzymes, Branched-Chain Aminotransferase (BCAT) and Branched-Chain α-Keto Acid Dehydrogenase (BCKD), in Human Pathophysiology

inferred← citeshttps://doi.org/10.3390/ijms242115999openalex-snapshot

Branched-Chain Amino Acid Assembly into Amyloid-like Fibrils Provides a New Paradigm for Maple Syrup Urine Disease Pathology

inferred← citeshttps://doi.org/10.3390/metabo12060527openalex-snapshot

Toxic Metabolites and Inborn Errors of Amino Acid Metabolism: What One Informs about the Other

inferred← citeshttps://doi.org/10.3390/nu15184023openalex-snapshot

Aspartic Acid in Health and Disease

inferred← citeshttps://doi.org/10.36948/ijfmr.2024.v06i04.26452openalex-snapshot

Review : Maple Syrup Urine Disease : A Rare Inherted Disorder of Amino Acid Metabolism

inferred← citeshttps://doi.org/10.6084/m9.figshare.19776320openalex-snapshot

Additional file 1 of Intravenous administration of a branched-chain amino-acid-free solution in children and adults with acute decompensation of maple syrup urine disease: a prospective multicentre observational study

inferred← citeshttps://doi.org/10.64898/2026.07.13.26357918openalex-snapshot

Associations between Elevated Circulating Amino Acid Levels and Brain Health: A Mendelian Randomization Study in the UK Biobank

inferred← citeshttps://doi.org/10.7744/kjoas.530209openalex-snapshot

Antioxidant and neuroprotective effects of Protaetia brevitarsis larvae against oxidative stress induced by hydrogen peroxide

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