Metabolic Dysfunctions in Amyotrophic Lateral Sclerosis Pathogenesis and Potential Metabolic Treatments

scientific article published on January 2016

Metabolic Dysfunctions in Amyotrophic Lateral Sclerosis Pathogenesis and Potential Metabolic Treatments is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.3389/FNINS.2016.00611
P932PMC publication ID5222822
P698PubMed publication ID28119559

P50authorTesfaye W TeferaQ88095214
Karin BorgesQ42409437
P2093author name stringKarin Borges
Tesfaye W Tefera
P2860cites workAnaplerotic triheptanoin diet enhances mitochondrial substrate use to remodel the metabolome and improve lifespan, motor function, and sociability in MeCP2-null miceQ21131923
Study of the ketogenic agent AC-1202 in mild to moderate Alzheimer's disease: a randomized, double-blind, placebo-controlled, multicenter trialQ21245063
A ketogenic diet as a potential novel therapeutic intervention in amyotrophic lateral sclerosisQ21262053
Pathways and genes differentially expressed in the motor cortex of patients with sporadic amyotrophic lateral sclerosisQ21266563
Aggregation and Motor Neuron Toxicity of an ALS-Linked SOD1 Mutant Independent from Wild-Type SOD1Q22299419
Heptanoate as a neural fuel: energetic and neurotransmitter precursors in normal and glucose transporter I-deficient (G1D) brainQ22337252
Ketogenic diet and other dietary treatments for epilepsyQ24186154
A ketogenic diet reduces amyloid beta 40 and 42 in a mouse model of Alzheimer's diseaseQ24816672
The microglial-motoneuron dialogue in ALSQ27000470
Cerebral metabolism following traumatic brain injury: new discoveries with implications for treatmentQ27023017
In Vivo imaging reveals distinct inflammatory activity of CNS microglia versus PNS macrophages in a mouse model for ALSQ27349669
Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosisQ28131805
Glutamate uptakeQ28190140
From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALSQ28206361
Design, power, and interpretation of studies in the standard murine model of ALSQ28268686
Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neuronsQ28298056
Long-term acetyl-L-carnitine treatment in Alzheimer's diseaseQ28333901
Abnormal excitatory amino acid metabolism in amyotrophic lateral sclerosisQ28335672
Effects of dichloroacetate on the metabolism of glucose, pyruvate, acetate, 3-hydroxybutyrate and palmitate in rat diaphragm and heart muscle in vitro and on extraction of glucose, lactate, pyruvate and free fatty acids by dog heart in vivoQ28364065
Triheptanoin improves brain energy metabolism in patients with Huntington diseaseQ28388261
Modulation of astrocytic mitochondrial function by dichloroacetate improves survival and motor performance in inherited amyotrophic lateral sclerosisQ28482111
Ketogenic diet improves forelimb motor function after spinal cord injury in rodentsQ28534800
High dietary fat induces NADPH oxidase-associated oxidative stress and inflammation in rat cerebral cortexQ28578642
ALS: a disease of motor neurons and their nonneuronal neighborsQ29618000
Onset and progression in inherited ALS determined by motor neurons and microgliaQ29619515
Glutamate uptake into astrocytes stimulates aerobic glycolysis: a mechanism coupling neuronal activity to glucose utilizationQ29620724
Mechanisms of ischemic neuroprotection by acetyl-L-carnitineQ30389339
Creatine monohydrate in ALS: effects on strength, fatigue, respiratory status and ALSFRS.Q30440341
Phase II trial of CoQ10 for ALS finds insufficient evidence to justify phase III.Q30494108
Glycolysis and the significance of lactate in traumatic brain injuryQ30938714
Selective targeting of a redox-active ubiquinone to mitochondria within cells: antioxidant and antiapoptotic propertiesQ31621261
Microarray analysis of the cellular pathways involved in the adaptation to and progression of motor neuron injury in the SOD1 G93A mouse model of familial ALS.Q33294971
Neuroprotective effects of the mitochondria-targeted antioxidant MitoQ in a model of inherited amyotrophic lateral sclerosisQ87384496
Clinical trials in amyotrophic lateral sclerosis: why so many negative trials and how can trials be improved?Q38260277
Dysregulation of astrocyte-motoneuron cross-talk in mutant superoxide dismutase 1-related amyotrophic lateral sclerosisQ38366741
AMPK Signalling and Defective Energy Metabolism in Amyotrophic Lateral SclerosisQ38552413
Cerebral Lactate Metabolism After Traumatic Brain InjuryQ38742790
A double-blind, placebo-controlled study to assess the mitochondria-targeted antioxidant MitoQ as a disease-modifying therapy in Parkinson's diseaseQ39110459
Oligodendrocyte dysfunction in the pathogenesis of amyotrophic lateral sclerosisQ39200143
Dexpramipexole versus placebo for patients with amyotrophic lateral sclerosis (EMPOWER): a randomised, double-blind, phase 3 trialQ39344567
A small molecule screen in stem-cell-derived motor neurons identifies a kinase inhibitor as a candidate therapeutic for ALS.Q39432513
Glycolytic oligodendrocytes maintain myelin and long-term axonal integrityQ39492965
Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis.Q40165089
Targeted antioxidative and neuroprotective properties of the dopamine agonist pramipexole and its nondopaminergic enantiomer SND919CL2x [(+)2-amino-4,5,6,7-tetrahydro-6-Lpropylamino-benzathiazole dihydrochloride].Q40368104
Aerobic production and utilization of lactate satisfy increased energy demands upon neuronal activation in hippocampal slices and provide neuroprotection against oxidative stressQ40385439
A clinical trial of creatine in ALS.Q40469445
Mitochondrial dysfunction in a cell culture model of familial amyotrophic lateral sclerosisQ40723426
Alpha-ketoglutarate and malate uptake and metabolism by synaptosomes: further evidence for an astrocyte-to-neuron metabolic shuttleQ41593514
Dietary anaplerotic therapy improves peripheral tissue energy metabolism in patients with Huntington's diseaseQ41734464
Mechanism of activation of pyruvate dehydrogenase by dichloroacetate and other halogenated carboxylic acidsQ41945381
Effects of dexpramipexole on brain mitochondrial conductances and cellular bioenergetic efficiencyQ42153700
Anticonvulsant effects of a triheptanoin diet in two mouse chronic seizure modelsQ42409348
Factors correlated with hypermetabolism in patients with amyotrophic lateral sclerosisQ42509201
Lactate reduces glutamate-induced neurotoxicity in rat cortexQ42514331
Lowered cerebral glucose utilization in amyotrophic lateral sclerosisQ42521169
Metabolic therapy with Deanna Protocol supplementation delays disease progression and extends survival in amyotrophic lateral sclerosis (ALS) mouse modelQ42749841
Short- and medium-chain fatty acids in energy metabolism: the cellular perspectiveQ42766173
Stimulation of oxidation of mitochondrial fatty acids and of acetate by acetylcarnitineQ42966552
Acetyl-L-carnitine reduces the infarct size and striatal glutamate outflow following focal cerebral ischemia in ratsQ42969544
Lactate is always the end product of glycolysisQ43166048
The human brain utilizes lactate via the tricarboxylic acid cycle: a 13C-labelled microdialysis and high-resolution nuclear magnetic resonance study.Q43287637
Neuroprotective role of lactate after cerebral ischemiaQ43292770
Dietary triheptanoin rescues oligodendrocyte loss, dysmyelination and motor function in the nur7 mouse model of Canavan diseaseQ43439006
Blockade of lactate transport exacerbates delayed neuronal damage in a rat model of cerebral ischemiaQ43548502
Increases in cortical glutamate concentrations in transgenic amyotrophic lateral sclerosis mice are attenuated by creatine supplementation.Q43572865
Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's diseaseQ43669934
The action of acetyl-L-carnitine on the neurotoxicity evoked by amyloid fragments and peroxide on primary rat cortical neuronesQ43681257
Effect of beta-hydroxybutyrate, a cerebral function improving agent, on cerebral hypoxia, anoxia and ischemia in mice and ratsQ43793315
Mitochondrial DNA and respiratory chain function in spinal cords of ALS patientsQ43883813
Lactate administration attenuates cognitive deficits following traumatic brain injuryQ43885977
Acetyl-L-carnitine shows neuroprotective and neurotrophic activity in primary culture of rat embryo motoneuronsQ44105695
Mitochondrial electron transport chain complex dysfunction in a transgenic mouse model for amyotrophic lateral sclerosisQ44187182
Different mechanisms contribute to motor cortex hyperexcitability in amyotrophic lateral sclerosisQ44205425
A randomized sequential trial of creatine in amyotrophic lateral sclerosisQ44383439
Effect of lactate therapy upon cognitive deficits after traumatic brain injury in the rat.Q44931184
Triheptanoin partially restores levels of tricarboxylic acid cycle intermediates in the mouse pilocarpine model of epilepsyQ45181504
Differential sirtuin expression patterns in amyotrophic lateral sclerosis (ALS) postmortem tissue: neuroprotective or neurotoxic properties of sirtuins in ALS?Q45388639
Cerebral metabolic effects of exogenous lactate supplementation on the injured human brainQ45840054
Triheptanoin supplementation to ketogenic diet curbs cognitive impairment in APP/PS1 mice used as a model of familial Alzheimer's disease.Q46056482
Mitochondria-targeted antioxidant effects of S(-) and R(+) pramipexoleQ33529864
Cognitive impairment following high fat diet consumption is associated with brain inflammation.Q33665160
Ethyl pyruvate protects against hypoxic-ischemic brain injury via anti-cell death and anti-inflammatory mechanismsQ33665291
The effects of a ketogenic diet on behavioral outcome after controlled cortical impact injury in the juvenile and adult rat.Q33744257
Metabolic and histologic effects of sodium pyruvate treatment in the rat after cortical contusion injuryQ33767257
Resveratrol ameliorates motor neuron degeneration and improves survival in SOD1(G93A) mouse model of amyotrophic lateral sclerosisQ33892886
Molecular factors underlying selective vulnerability of motor neurons to neurodegeneration in amyotrophic lateral sclerosisQ33906246
Creatine and its potential therapeutic value for targeting cellular energy impairment in neurodegenerative diseasesQ33917550
Oligodendroglia metabolically support axons and contribute to neurodegeneration.Q34032818
Neuroprotective effects of pyruvate following NMDA-mediated excitotoxic insults in hippocampal slicesQ34034916
Cytoskeletal abnormalities in amyotrophic lateral sclerosis: beneficial or detrimental effects?Q34088731
Beneficial effects of sodium or ethyl pyruvate after traumatic brain injury in the ratQ34126225
Body mass index, not dyslipidemia, is an independent predictor of survival in amyotrophic lateral sclerosisQ34187004
Hypercaloric enteral nutrition in patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled phase 2 trialQ34251334
Clinical pharmacology of the dietary supplement creatine monohydrate.Q34253434
Selective loss of glial glutamate transporter GLT‐1 in amyotrophic lateral sclerosisQ34300016
Sirtuins as therapeutic targets of ALS.Q34357662
Nutritional status of patients with amyotrophic lateral sclerosis: relation to the proximity of deathQ34375696
Caprylic triglyceride as a novel therapeutic approach to effectively improve the performance and attenuate the symptoms due to the motor neuron loss in ALS diseaseQ34474226
Oxidative stress in ALS: a mechanism of neurodegeneration and a therapeutic targetQ34567689
Energy intake and amyotrophic lateral sclerosisQ34582888
Identification and characterization of cholest-4-en-3-one, oxime (TRO19622), a novel drug candidate for amyotrophic lateral sclerosisQ34627577
Dexpramipexole is ineffective in two models of ALS related neurodegenerationQ34744144
Neuroprotection by acetyl-L-carnitine after traumatic injury to the immature rat brainQ34777969
Weight loss, dysphagia and supplement intake in patients with amyotrophic lateral sclerosis (ALS): impact on quality of life and therapeutic optionsQ34815254
Dichloroacetate effects on glucose and lactate oxidation by neurons and astroglia in vitro and on glucose utilization by brain in vivoQ34961411
Gene expression signatures in motor neurone disease fibroblasts reveal dysregulation of metabolism, hypoxia-response and RNA processing functionsQ35086167
Wild-type microglia extend survival in PU.1 knockout mice with familial amyotrophic lateral sclerosisQ35094527
Ketogenic diet prevents alterations in brain metabolism in young but not adult rats after traumatic brain injury.Q35212553
Triheptanoin for glucose transporter type I deficiency (G1D): modulation of human ictogenesis, cerebral metabolic rate, and cognitive indices by a food supplementQ35223033
An imbalance between excitatory and inhibitory neurotransmitters in amyotrophic lateral sclerosis revealed by use of 3-T proton magnetic resonance spectroscopy.Q35246605
Phase 1 trial of dichloroacetate (DCA) in adults with recurrent malignant brain tumorsQ35684292
Early and progressive impairment of spinal blood flow-glucose metabolism coupling in motor neuron degeneration of ALS model miceQ35800499
A metabolic switch toward lipid use in glycolytic muscle is an early pathologic event in a mouse model of amyotrophic lateral sclerosisQ35822554
D-beta-hydroxybutyrate rescues mitochondrial respiration and mitigates features of Parkinson diseaseQ35919815
Endogenous Nutritive Support after Traumatic Brain Injury: Peripheral Lactate Production for Glucose Supply via GluconeogenesisQ35932745
Lactate: brain fuel in human traumatic brain injury: a comparison with normal healthy control subjects.Q35932802
Decreased motor cortex γ-aminobutyric acid in amyotrophic lateral sclerosis.Q35948547
Exogenous lactate infusion improved neurocognitive function of patients with mild traumatic brain injuryQ35983324
Triheptanoin Protects Motor Neurons and Delays the Onset of Motor Symptoms in a Mouse Model of Amyotrophic Lateral SclerosisQ36114275
Coenzyme Q10 administration increases brain mitochondrial concentrations and exerts neuroprotective effectsQ36225925
A probable dual mode of action for both L- and D-lactate neuroprotection in cerebral ischemiaQ36267408
Anaplerotic molecules: current and futureQ36501322
The role of excitotoxicity in the pathogenesis of amyotrophic lateral sclerosis.Q36522329
L-Lactate protects neurons against excitotoxicity: implication of an ATP-mediated signaling cascade.Q36596141
Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice.Q36677109
Sodium pyruvate reduces hypoxic-ischemic injury to neonatal rat brainQ36682284
Lost in translation: treatment trials in the SOD1 mouse and in human ALS.Q36735871
Premorbid body mass index and risk of amyotrophic lateral sclerosisQ36737158
Triheptanoin dramatically reduces paroxysmal motor disorder in patients with GLUT1 deficiencyQ36859501
MitoQ--a mitochondria-targeted antioxidant.Q36887240
High-Fat Diet Induced Anxiety and Anhedonia: Impact on Brain Homeostasis and InflammationQ36906680
Reduced activity of AMP-activated protein kinase protects against genetic models of motor neuron diseaseQ37093737
KNS-760704 [(6R)-4,5,6,7-tetrahydro-N6-propyl-2, 6-benzothiazole-diamine dihydrochloride monohydrate] for the treatment of amyotrophic lateral sclerosis.Q37272764
Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse modelQ37285915
Ketosis and brain handling of glutamate, glutamine, and GABAQ37294731
Mitochondrial dysfunction in SOD1G93A-bearing astrocytes promotes motor neuron degeneration: prevention by mitochondrial-targeted antioxidantsQ37352307
Alterations of hippocampal glucose metabolism by even versus uneven medium chain triglycerides.Q37459904
Resveratrol improves motoneuron function and extends survival in SOD1(G93A) ALS mice.Q37717433
Energy metabolism in amyotrophic lateral sclerosisQ37805077
Mitochondrial dysfunction in ALS.Q37914353
The changing scene of amyotrophic lateral sclerosisQ38086771
ALS Untangled No. 20: the Deanna protocolQ38103571
Oligodendroglia: metabolic supporters of axonsQ38132499
Body mass index and dietary intervention: implications for prognosis of amyotrophic lateral sclerosis.Q38195974
Half-molar sodium lactate infusion to prevent intracranial hypertensive episodes in severe traumatic brain injured patients: a randomized controlled trialQ46067965
Ketogenic diet reduces cytochrome c release and cellular apoptosis following traumatic brain injury in juvenile rats.Q46129124
Wild-Type Nonneuronal Cells Extend Survival of SOD1 Mutant Motor Neurons in ALS MiceQ46254778
Randomized double-blind placebo-controlled trial of acetyl-L-carnitine for ALS.Q46281670
Sodium lactate versus mannitol in the treatment of intracranial hypertensive episodes in severe traumatic brain-injured patientsQ46354513
Protection by exogenous pyruvate through a mechanism related to monocarboxylate transporters against cell death induced by hydrogen peroxide in cultured rat cortical neurons.Q46386518
Pyruvate prevents the development of age-dependent cognitive deficits in a mouse model of Alzheimer's disease without reducing amyloid and tau pathologyQ46457932
Age-dependent reduction of cortical contusion volume by ketones after traumatic brain injuryQ46492468
Lactate: a major and crucial player in normal function of both muscle and brainQ46565218
Inhibition of the cerebral ischemic injury by ethyl pyruvate with a wide therapeutic windowQ46686089
The neuroprotective effect of lactate is not due to improved glutamate uptake after controlled cortical impact in ratsQ47269521
The protective effect of the ketogenic diet on traumatic brain injury-induced cell death in juvenile ratsQ47902341
Pyruvate and lactate protect striatal neurons against N-methyl-D-aspartate-induced neurotoxicityQ48098641
Neuroprotective effects of lactate in brain ischemia: dependence on anesthetic drugsQ48100624
New evidence of neuroprotection by lactate after transient focal cerebral ischaemia: extended benefit after intracerebroventricular injection and efficacy of intravenous administrationQ48115530
Reduced GABAergic inhibition explains cortical hyperexcitability in the wobbler mouse model of ALS.Q48121748
D-beta-hydroxybutyrate prevents glutamate-mediated lipoperoxidation and neuronal damage elicited during glycolysis inhibition in vivoQ48364448
The expression of the glial glutamate transporter protein EAAT2 in motor neuron disease: an immunohistochemical study.Q48378456
Novel threshold tracking techniques suggest that cortical hyperexcitability is an early feature of motor neuron diseaseQ48474459
Inverse relationship between brain glucose and ketone metabolism in adults during short-term moderate dietary ketosis: A dual tracer quantitative positron emission tomography study.Q48513277
Frontal lobe function in amyotrophic lateral sclerosis: a neuropsychologic and positron emission tomography studyQ48535842
Pyruvate protects neurons against hydrogen peroxide-induced toxicityQ48579857
Bioenergetic abnormalities in discrete cerebral motor pathways presage spinal cord pathology in the G93A SOD1 mouse model of ALS.Q48582883
Anticonvulsant profile of caprylic acid, a main constituent of the medium-chain triglyceride (MCT) ketogenic diet, in mice.Q48735249
Cortical hyperexcitability may precede the onset of familial amyotrophic lateral sclerosisQ48881997
Ethyl pyruvate rescues nigrostriatal dopaminergic neurons by regulating glial activation in a mouse model of Parkinson's diseaseQ49153506
Neuroprotective effects of α-ketoglutarate and ethyl pyruvate against motor dysfunction and oxidative changes caused by repeated 1-methyl-4-phenyl-1,2,3,6 tetrahydropyridine exposure in mice.Q50861907
Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice.Q51714635
Hypermetabolism in ALS patients: an early and persistent phenomenon.Q51755449
Clinical and neurochemical effects of acetyl-L-carnitine in Alzheimer's disease.Q53202071
Acetyl-L-carnitineameliorates mitochondrial damage and apoptosis following spinal cord injury in rats.Q53466722
Pyruvate slows disease progression in a G93A SOD1 mutant transgenic mouse model.Q53582566
Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis.Q55032889
Amyotrophic lateral sclerosisQ55877676
The effects of dexpramipexole (KNS-760704) in individuals with amyotrophic lateral sclerosisQ56773744
Alteration of nutritional status at diagnosis is a prognostic factor for survival of amyotrophic lateral sclerosis patientsQ56839377
A phase II−III trial of olesoxime in subjects with amyotrophic lateral sclerosisQ57666978
Preclinical research: Make mouse studies workQ59058234
The metabolic signature of C9ORF72-related ALS: FDG PET comparison with nonmutated patientsQ59901143
Mutant TDP-43 deregulates AMPK activation by PP2A in ALS modelsQ60552719
Triheptanoin in acute mouse seizure modelsQ62785908
Antioxidant strategies based on tomato-enriched food or pyruvate do not affect disease onset and survival in an animal model of amyotrophic lateral sclerosisQ63410203
Cortical motor-sensory hypometabolism in amyotrophic lateral sclerosis: a PET studyQ68095604
Acetyl-L-carnitine attenuates neuronal damage in gerbils with transient forebrain ischemia only when given before the insultQ70921038
A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study GroupQ72759362
Avid Na+-dependent, high-affinity uptake of alpha-ketoglutarate by nerve terminal enriched material from mouse cerebellumQ72888128
Neuroprotective effects of acetyl-L-carnitine after stroke in ratsQ73396042
Excitatory amino acid transporter 1 and 2 immunoreactivity in the spinal cord in amyotrophic lateral sclerosisQ74237337
EFFECTS OF CARNITINE ON ACETYL-COA OXIDATION BY HEART MUSCLE MITOCHONDRIAQ76846436
Olesoxime delays muscle denervation, astrogliosis, microglial activation and motoneuron death in an ALS mouse modelQ83522395
P921main subjectamyotrophic lateral sclerosisQ206901
P304page(s)611
P577publication date2016-01-01
P1433published inFrontiers in NeuroscienceQ2177807
P1476titleMetabolic Dysfunctions in Amyotrophic Lateral Sclerosis Pathogenesis and Potential Metabolic Treatments
P478volume10

Reverse relations

cites work (P2860)
Q64230186Astrocyte adenosine deaminase loss increases motor neuron toxicity in amyotrophic lateral sclerosis
Q97692894Brain energy rescue: an emerging therapeutic concept for neurodegenerative disorders of ageing
Q41032768Brugada syndrome in a patient with amyotrophic lateral sclerosis: a case report
Q33729341Comparison of Sirtuin 3 Levels in ALS and Huntington's Disease-Differential Effects in Human Tissue Samples vs. Transgenic Mouse Models
Q42377167Genetic induction of hypometabolism by ablation of MC4R does not suppress ALS-like phenotypes in the G93A mutant SOD1 mouse model.
Q50118955Lymphoblastoid cell lines as a model to understand amyotrophic lateral sclerosis disease mechanisms
Q89823775Medium Chain Triglycerides Modulate the Ketogenic Effect of a Metabolic Switch
Q60949835Metabolomics Biomarkers: A Strategy Toward Therapeutics Improvement in ALS
Q62785679Neuronal glucose metabolism is impaired while astrocytic TCA cycling is unaffected at symptomatic stages in the hSOD1G93A mouse model of amyotrophic lateral sclerosis
Q50091767Quantitative susceptibility mapping of the motor cortex: a comparison of susceptibility among patients with amyotrophic lateral sclerosis, cerebrovascular disease, and healthy controls
Q61817166Role of Ketogenic Diets in Neurodegenerative Diseases (Alzheimer's Disease and Parkinson's Disease)

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