Biomarkers of Metabolism in Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the deterioration of motor neurons. However, this complex disease extends beyond the boundaries of the central nervous system, with metabolic alterations being observed at the systemic and cellular level. While the number of studies that assess the role and impact of metabolic perturbations in ALS is rapidly increasing, the use of metabolism biomarkers in ALS remains largely underinvestigated. In this review, we discuss current and potential metabolism biomarkers in the context of ALS. Of those for which data does exist, there is limited insight provided by individual markers, with specificity for disease, and lack of reproducibility and efficacy in informing prognosis being the largest drawbacks. However, given the array of metabolic markers available, the potential exists for a panel of metabolism biomarkers, which may complement other current biomarkers (including neurophysiology, imaging, as well as CSF, blood and urine markers) to overturn these limitations and give rise to new diagnostic and prognostic indicators.

• Publication year

  2019

• Venue

  Frontiers in Neurology

• Publication date

  2019-03-18

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.3389/fneur.2019.00191PMID 30936848PMCID 6431787
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Early weight loss in amyotrophic lateral sclerosis: outcome relevance and clinical correlates in a population-based cohort

  2019cited by this paper
• Validation of the Method

  2019cited by this paper
• The multistep hypothesis of ALS revisited

  2018cited by this paper
• Hypermetabolism in ALS is associated with greater functional decline and shorter survival

  2018cited by this paper
• Interplay between spinal cord and cerebral cortex metabolism in amyotrophic lateral sclerosis

  2018cited by this paper
• Hypermetabolism is a deleterious prognostic factor in patients with amyotrophic lateral sclerosis

  2018cited by this paper
• Low IDL-B and high LDL-1 subfraction levels in serum of ALS patients.

  2017cited by this paper
• The vicious circle of hypometabolism in neurodegenerative diseases: Ways and mechanisms of metabolic correction

  2017cited by this paper
• Percutaneous endoscopic gastrostomy, body weight loss and survival in amyotrophic lateral sclerosis: a population-based registry study

  2017cited by this paper
• Lipidomics Reveals Cerebrospinal-Fluid Signatures of ALS

  2017cited by this paper
• Body Mass Index and Amyotrophic Lateral Sclerosis: A Study of US Military Veterans

  2017cited by this paper
• Flow-metabolism uncoupling in the cervical spinal cord of ALS patients

  2017cited by this paper
• Correlation of Creatine Kinase Levels with Clinical Features and Survival in Amyotrophic Lateral Sclerosis

  2017cited by this paper
• Cerebral hypoperfusion and glucose hypometabolism: Key pathophysiological modulators promote neurodegeneration, cognitive impairment, and Alzheimer's disease

  2017cited by this paper
• Anthropometric measures are not accurate predictors of fat mass in ALS

  2017cited by this paper
• Blood biomarkers of carbohydrate, lipid, and apolipoprotein metabolisms and risk of amyotrophic lateral sclerosis: A more than 20‐year follow‐up of the Swedish AMORIS cohort

  2017cited by this paper
• Amyotrophic Lateral Sclerosis.

  2017cited by this paper
• Life course body mass index and risk and prognosis of amyotrophic lateral sclerosis: results from the ALS registry Swabia

  2017cited by this paper
• Exploring targets and therapies for amyotrophic lateral sclerosis: current insights into dietary interventions

  2017cited by this paper
• Changes in Socioeconomic Inequality of Low Birth Weight and Macrosomia in Shaanxi Province of Northwest China, 2010–2013

  2016cited by this paper
• Defective cholesterol metabolism in amyotrophic lateral sclerosis[S]

  2016influential reference
• Creatine kinase enzyme level correlates positively with serum creatinine and lean body mass, and is a prognostic factor for survival in amyotrophic lateral sclerosis

  2016cited by this paper
• p75 Neurotrophin Receptor Regulates Energy Balance in Obesity.

  2016cited by this paper
• A PET/CT approach to spinal cord metabolism in amyotrophic lateral sclerosis

  2016cited by this paper
• Insulin Resistance and Neurodegeneration: Progress Towards the Development of New Therapeutics for Alzheimer’s Disease

  2016cited by this paper
• The role of the SenseWear device and ghrelin for metabolism in amyotrophic lateral sclerosis

  2016cited by this paper
• MR Imaging of Brachial Plexus and Limb-Girdle Muscles in Patients with Amyotrophic Lateral Sclerosis.

  2016cited by this paper
• Integrated Network Analysis Reveals an Association between Plasma Mannose Levels and Insulin Resistance.

  2016cited by this paper
• The longitudinal cerebrospinal fluid metabolomic profile of amyotrophic lateral sclerosis

  2015cited by this paper
• Association between nutritional status and disease severity using the amyotrophic lateral sclerosis (ALS) functional rating scale in ALS patients.

  2015cited by this paper
• Altered expression of metabolic proteins and adipokines in patients with amyotrophic lateral sclerosis.

  2015influential reference
• Factors Predicting Survival in ALS Patients - Data from a Population-Based Registry in Rhineland-Palatinate, Germany

  2015cited by this paper
• The serum lipid profiles of amyotrophic lateral sclerosis patients: A study from south-west China and a meta-analysis

  2015cited by this paper
• Relationship of creatine kinase to body composition, disease state, and longevity in ALS

  2015cited by this paper
• Nutritional assessment of amyotrophic lateral sclerosis in routine practice: Value of weighing and bioelectrical impedance analysis

  2015cited by this paper
• Effect of lipid profile on prognosis in the patients with amyotrophic lateral sclerosis: Insights from the olesoxime clinical trial

  2015cited by this paper
• Plasma cortisol level in amyotrophic lateral sclerosis.

  2015cited by this paper
• A metabolic switch toward lipid use in glycolytic muscle is an early pathologic event in a mouse model of amyotrophic lateral sclerosis

  2015cited by this paper
• Factors predicting one-year mortality in amyotrophic lateral sclerosis patients – data from a population-based registry

  2014cited by this paper
• Functional pattern of brain FDG-PET in amyotrophic lateral sclerosis

  2014cited by this paper
• Analysis of amyotrophic lateral sclerosis as a multistep process: a population-based modelling study

  2014cited by this paper
• The Extracellular Domain of Neurotrophin Receptor p75 as a Candidate Biomarker for Amyotrophic Lateral Sclerosis

  2014cited by this paper
• Premorbid body mass index and risk of amyotrophic lateral sclerosis

  2013cited by this paper
• NMR-based Metabonomics of Cerebrospinal Fluid Applied to Amyotrophic Lateral Sclerosis

  2013cited by this paper
• Body mass index (BMI) as predictor of ALSFRS-R score decline in ALS patients

  2013cited by this paper
• of amyotrophic lateral sclerosis

  2013cited by this paper
• Rates of progression of weight and forced vital capacity as relevant measurement to adapt amyotrophic lateral sclerosis management for patient Result of a French multicentre cohort survey.

  2013cited by this paper
• Adipose Tissue Distribution Predicts Survival in Amyotrophic Lateral Sclerosis

  2013cited by this paper
• Incidence of and risk factors for Motor Neurone Disease in UK women: a prospective study

  2012cited by this paper
• Relationships between disease progression and serum levels of lipid, urate, creatinine and ferritin in Japanese patients with amyotrophic lateral sclerosis: a cross-sectional study.

  2012cited by this paper
• p75 neurotrophin receptor regulates glucose homeostasis and insulin sensitivity

  2012cited by this paper
• Biochemical alterations associated with ALS

  2012cited by this paper
• Reduction rate of body mass index predicts prognosis for survival in amyotrophic lateral sclerosis: A multicenter study in Japan

  2012cited by this paper
• Body mass index, not dyslipidemia, is an independent predictor of survival in amyotrophic lateral sclerosis

  2011cited by this paper
• Patients with elevated triglyceride and cholesterol serum levels have a prolonged survival in amyotrophic lateral sclerosis

  2011cited by this paper
• Energy metabolism in amyotrophic lateral sclerosis.

  2011cited by this paper
• A randomized controlled clinical trial of growth hormone in amyotrophic lateral sclerosis: clinical, neuroimaging, and hormonal results

  2011cited by this paper
• Alteration of nutritional status at diagnosis is a prognostic factor for survival of amyotrophic lateral sclerosis patients

  2010influential reference
• Impaired glucose tolerance in patients with amyotrophic lateral sclerosis

  2010cited by this paper
• The GH–IGF system in amyotrophic lateral sclerosis: correlations between pituitary GH secretion capacity, insulin‐like growth factors and clinical features

  2010cited by this paper
• The stripe of primary lateral sclerosis: focal primary motor cortex hypometabolism seen on fluorodeoxyglucose F18 positron emission tomography.

  2010cited by this paper
• 1H-NMR-Based Metabolomic Profiling of CSF in Early Amyotrophic Lateral Sclerosis

  2010cited by this paper
• Skeletal muscle-restricted expression of human SOD1 causes motor neuron degeneration in transgenic mice.

  2010cited by this paper
• Metabolomic analysis of serum by (1) H NMR spectroscopy in amyotrophic lateral sclerosis.

  2010cited by this paper
• A decrease in body mass index is associated with faster progression of motor symptoms and shorter survival in ALS

  2010influential reference
• Lower serum lipid levels are related to respiratory impairment in patients with ALS

  2009cited by this paper
• Amyotrophic lateral sclerosis

  2009cited by this paper
• Muscle Mitochondrial Uncoupling Dismantles Neuromuscular Junction and Triggers Distal Degeneration of Motor Neurons

  2009cited by this paper
• The Vote of Acute Medical Inpatients

  2009cited by this paper
• Skeletal muscle is a primary target of SOD1G93A-mediated toxicity.

  2008cited by this paper
• Dyslipidemia is a protective factor in amyotrophic lateral sclerosis

  2008cited by this paper
• Impaired Fasting Glucose and Impaired Glucose Tolerance

  2007cited by this paper
• Amyotrophic lateral sclerosis

  2007cited by this paper
• Growth hormone secretion is impaired in amyotrophic lateral sclerosis

  2006cited by this paper
• Muscular mitochondrial function in amyotrophic lateral sclerosis is progressively altered as the disease develops: a temporal study in man.

  2006cited by this paper
• Comparison of the growth hormone, IGF‐1 and insulin in cerebrospinal fluid and serum between patients with motor neuron disease and healthy controls

  2006influential reference
• Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse model.

  2004cited by this paper
• Up‐regulation of mitochondrial uncoupling protein 3 reveals an early muscular metabolic defect in amyotrophic lateral sclerosis

  2003cited by this paper
• Adrenal dysregulation in amyotrophic lateral sclerosis

  2003cited by this paper
• Creatine kinase activity in amyotrophic lateral sclerosis patients

  2003cited by this paper
• Lipid-lowering drugs and mitochondrial function: effects of HMG-CoA reductase inhibitors on serum ubiquinone and blood lactate/pyruvate ratio.

  2003cited by this paper
• Increased creatine kinase and spontaneous activity on electromyography, in amyotrophic lateral sclerosis.

  2003cited by this paper
• Role of fluorodeoxyglucose positron emission tomography in the diagnosis of neurosarcoidosis.

  2002cited by this paper
• Evidence that accumulation of ceramides and cholesterol esters mediates oxidative stress–induced death of motor neurons in amyotrophic lateral sclerosis

  2002cited by this paper
• Glucose metabolism in patients with acute myocardial infarction and no previous diagnosis of diabetes mellitus: a prospective study.

  2002cited by this paper
• Premorbid weight, body mass, and varsity athletics in ALS

  2002cited by this paper
• Mitochondrial DNA abnormalities in skeletal muscle of patients with sporadic amyotrophic lateral sclerosis.

  2000cited by this paper
• Nutritional assessment and survival in ALS patients

  2000cited by this paper
• Nutritional assessment and survival in ALS patients.

  2000cited by this paper
• Prognostic indicators of survival in ALS. ALS CNTF Treatment Study Group.

  1998cited by this paper
• Impairment of mitochondrial function in skeletal muscle of patients with amyotrophic lateral sclerosis.

  1998cited by this paper
• Creatine kinase values in amyotrophic lateral sclerosis.

  1998cited by this paper
• Prognostic indicators of survival in ALS

  1998cited by this paper
• Metabolic control and metabolic capacity: two aspects of creatine kinase functioning in the cells.

  1996cited by this paper
• Nutritional status of patients with amyotrophic lateral sclerosis: relation to the proximity of death.

  1996cited by this paper
• The p75 neurotrophin receptor.

  1994cited by this paper
• Frontal lobe function in amyotrophic lateral sclerosis: a neuropsychologic and positron emission tomography study

  1992cited by this paper
• Implications for care.

  1990cited by this paper
• Reduction in insulin receptors in amyotrophic lateral sclerosis correlates with reduced insulin sensitivity

  1987cited by this paper
• Lowered cerebral glucose utilization in amyotrophic lateral sclerosis

  1987cited by this paper

• Mendelian randomization of plasma proteomics identifies novel ALS-associated proteins and their GO enrichment and KEGG pathway analyses

  2025cites this paper
• Decremental response in patients with amyotrophic lateral sclerosis during repetitive nerve stimulation and its relationships with impaired homeostasis

  2025cites this paper
• Biomarkers in ALS trials: from discovery to clinical utility

  2025cites this paper
• Unraveling the Heterogeneity of ALS—A Call to Redefine Patient Stratification for Better Outcomes in Clinical Trials

  2024cites this paper
• Fluid biomarkers for amyotrophic lateral sclerosis: a review

  2024cites this paper
• Neutrophil-to-lymphocyte ratio at diagnosis as a biomarker for survival of amyotrophic lateral sclerosis

  2024cites this paper
• Amyotrophic lateral sclerosis stratification: unveiling patterns with virome, inflammation, and metabolism molecules

  2024cites this paper
• Evolving markers in amyotrophic lateral sclerosis.

  2023cites this paper
• A Diagnostic Gene-Expression Signature in Fibroblasts of Amyotrophic Lateral Sclerosis

  2023cites this paper
• Biofluid biomarkers of amyotrophic lateral sclerosis

  2022cites this paper
• Neuroimmunometabolism: A New Pathological Nexus Underlying Neurodegenerative Disorders

  2022cites this paper
• Mutation‐specific metabolic profiles in presymptomatic amyotrophic lateral sclerosis

  2022cites this paper
• Amyotrophic lateral sclerosis (ALS) and the endocrine system: Are there any further ties to be explored?

  2021cites this paper
• Ghrelin as a treatment for amyotrophic lateral sclerosis

  2021cites this paper
• Targeting Adenosine Receptors in Neurological Diseases

  2021cites this paper
• Hypothalamus and weight loss in amyotrophic lateral sclerosis.

  2021cites this paper
• Transcriptome analysis using patient iPSC-derived skeletal myocytes: Bet1L as a new molecule possibly linked to neuromuscular degeneration in ALS.

  2021cites this paper
• Metabolomic Biomarkers Are Associated With Area of the Pons in Fragile X Premutation Carriers at Risk for Developing FXTAS

  2021cites this paper
• Prognostic prediction by hypermetabolism varies depending on the nutritional status in early amyotrophic lateral sclerosis

  2021cites this paper
• Low plasma hyaluronan is associated with faster functional decline in patients with amyotrophic lateral sclerosis

  2021cites this paper
• Repurposing of Trimetazidine for amyotrophic lateral sclerosis: A study in SOD1G93A mice

  2021cites this paper
• Understanding and managing metabolic dysfunction in Amyotrophic Lateral Sclerosis

  2020cites this paper
• Blood biomarkers and prognosis of amyotrophic lateral sclerosis

  2020cites this paper
• Peripheral Glycolysis in Neurodegenerative Diseases

  2020cites this paper
• Genome-wide Meta-analysis Finds the ACSL5-ZDHHC6 Locus Is Associated with ALS and Links Weight Loss to the Disease Genetics

  2020cites this paper
• Progression and survival of patients with motor neuron disease relative to their fecal microbiota

  2020cites this paper
• Targeting Purinergic Signaling and Cell Therapy in Cardiovascular and Neurodegenerative Diseases.

  2019cites this paper
• Existing and Emerging Metabolomic Tools for ALS Research

  2019cites this paper
• Theme 10 Disease stratification and phenotyping of patients

  2019cites this paper