Disease-modifying effects of metabolic perturbations in ALS/FTLD (Q59794101)

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12 April 2020

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4 February 2021

Disease-modifying effects of metabolic perturbations in ALS/FTLD (English)

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:30509290%20AND%20SRC:MED&resulttype=core&format=json

amyotrophic lateral sclerosis

12 April 2020

main subject

molecular biology

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25 April 2019

frontotemporal dementia

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frontotemporal lobar degeneration

25 April 2019

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4 February 2021

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4 February 2021

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author name string

Romesa Khan

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2

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12 April 2020

Magdalini Polymenidou

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3

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12 April 2020

Paul E Schulz

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4

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4 December 2018

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12 April 2020

4 February 2021

Molecular Neurodegeneration

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25 April 2019

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4 February 2021

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1

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Obesity and vascular risk factors at midlife and the risk of dementia and Alzheimer disease

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A ketogenic diet as a potential novel therapeutic intervention in amyotrophic lateral sclerosis

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Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21

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Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17

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Transient caloric restriction in early adulthood hastens disease endpoint in male, but not female, Cu/Zn-SOD mutant G93A mice.

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Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

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Mitochondrial Respiration Is Decreased in Skeletal Muscle of Patients With Type 2 Diabetes

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Body mass index in midlife and risk of Alzheimer disease and vascular dementia

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Increased peripheral lipid clearance in an animal model of amyotrophic lateral sclerosis

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The neuropathology and clinical phenotype of FTD with progranulin mutations

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Statins, neuromuscular degenerative disease and an amyotrophic lateral sclerosis-like syndrome: an analysis of individual case safety reports from vigibase

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Cognitive impairment in familial ALS.

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Dyslipidemia is a protective factor in amyotrophic lateral sclerosis

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TDP-43 A315T mutation in familial motor neuron disease

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12 December 2020

TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis

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12 December 2020

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

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12 December 2020

Progranulin functions as a neurotrophic factor to regulate neurite outgrowth and enhance neuronal survival

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12 December 2020

TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis

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12 December 2020

TDP-43 mutation in familial amyotrophic lateral sclerosis

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12 December 2020

Emerging role for AS160/TBC1D4 and TBC1D1 in the regulation of GLUT4 traffic

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12 December 2020

IGF1 is a common target gene of Ewing's sarcoma fusion proteins in mesenchymal progenitor cells.

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12 December 2020

Influence of statins treatment on survival in patients with amyotrophic lateral sclerosis

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12 December 2020

Metabolomic analysis and signatures in motor neuron disease

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12 December 2020

P bodies promote stress granule assembly in Saccharomyces cerevisiae

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12 December 2020

ALS in Italian professional soccer players: the risk is still present and could be soccer-specific.

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12 December 2020

Lifestyle factors and risk of amyotrophic lateral sclerosis: a case-control study in Japan

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12 December 2020

Diabetes preceding Parkinson's disease onset. A case-control study.

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12 December 2020

Mitochondrial calcium as a key regulator of mitochondrial ATP production in mammalian cells

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12 December 2020

Association between late-life body mass index and dementia: The Kame Project

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12 December 2020

Understanding the Warburg effect: the metabolic requirements of cell proliferation

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12 December 2020

Stable interference of EWS-FLI1 in an Ewing sarcoma cell line impairs IGF-1/IGF-1R signalling and reveals TOPK as a new target

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12 December 2020

1 reference

12 December 2020

Analysis of Ewing sarcoma (EWS)-binding proteins: interaction with hnRNP M, U, and RNA-helicases p68/72 within protein-RNA complexes

1 reference

12 December 2020

Pre-symptomatic detection of chronic motor deficits and genotype prediction in congenic B6.SOD1(G93A) ALS mouse model.

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12 December 2020

Progranulin is expressed within motor neurons and promotes neuronal cell survival

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12 December 2020

ALS disease onset may occur later in patients with pre-morbid diabetes mellitus

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12 December 2020

TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration

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12 December 2020

Caloric restriction shortens lifespan through an increase in lipid peroxidation, inflammation and apoptosis in the G93A mouse, an animal model of ALS.

1 reference

12 December 2020

Amyotrophic lateral sclerosis, physical exercise, trauma and sports: results of a population-based pilot case-control study

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12 December 2020

A decrease in body mass index is associated with faster progression of motor symptoms and shorter survival in ALS

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12 December 2020

Statin use and risk of amyotrophic lateral sclerosis and other motor neuron disorders

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12 December 2020

Mutant TAR DNA-binding protein-43 induces oxidative injury in motor neuron-like cell

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12 December 2020

Progressive motor weakness in transgenic mice expressing human TDP-43.

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12 December 2020

Deletion of TDP-43 down-regulates Tbc1d1, a gene linked to obesity, and alters body fat metabolism

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12 December 2020

Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice

1 reference

12 December 2020

TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia

1 reference

12 December 2020

Gender-based effect of statins on functional decline in amyotrophic lateral sclerosis

1 reference

12 December 2020

Patients with elevated triglyceride and cholesterol serum levels have a prolonged survival in amyotrophic lateral sclerosis.

1 reference

12 December 2020

CC3/TIP30 regulates metabolic adaptation of tumor cells to glucose limitation

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12 December 2020

Huntington's disease: a clinical review

1 reference

12 December 2020

Abnormal exocytotic release of glutamate in a mouse model of amyotrophic lateral sclerosis.

1 reference

12 December 2020

PGC-1α regulates translocated in liposarcoma activity: role in oxidative stress gene expression

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12 December 2020

Caloric restriction and resveratrol promote longevity through the Sirtuin-1-dependent induction of autophagy

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12 December 2020

AMPK phosphorylates and inhibits SREBP activity to attenuate hepatic steatosis and atherosclerosis in diet-induced insulin-resistant mice

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12 December 2020

Amyotrophic lateral sclerosis is associated with hypolipidemia at the presymptomatic stage in mice

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12 December 2020

Beneficial vascular risk profile is associated with amyotrophic lateral sclerosis

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12 December 2020

Medicine. Progranulin resolves inflammation

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12 December 2020

Body mass index, not dyslipidemia, is an independent predictor of survival in amyotrophic lateral sclerosis

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12 December 2020

A novel oncogenic mechanism in Ewing sarcoma involving IGF pathway targeting by EWS/Fli1-regulated microRNAs

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12 December 2020

Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS

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12 December 2020

A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD

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12 December 2020

The seeds of neurodegeneration: prion-like spreading in ALS

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12 December 2020

Cognitive decline typical of frontotemporal lobar degeneration in transgenic mice expressing the 25-kDa C-terminal fragment of TDP-43.

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12 December 2020

Biochemical alterations associated with ALS.

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12 December 2020

PGRN is a key adipokine mediating high fat diet-induced insulin resistance and obesity through IL-6 in adipose tissue

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12 December 2020

FET proteins in frontotemporal dementia and amyotrophic lateral sclerosis

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12 December 2020

Tunicamycin produces TDP-43 cytoplasmic inclusions in cultured brain organotypic slices

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12 December 2020

Medical and environmental risk factors associated with frontotemporal dementia: a case-control study in a veteran population.

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12 December 2020

Reduction rate of body mass index predicts prognosis for survival in amyotrophic lateral sclerosis: a multicenter study in Japan

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12 December 2020

A randomized, double blind, placebo-controlled trial of pioglitazone in combination with riluzole in amyotrophic lateral sclerosis

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12 December 2020

Is hyperlipidemia correlated with longer survival in patients with amyotrophic lateral sclerosis?

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12 December 2020

Clinical features and lifestyle of patients with amyotrophic lateral sclerosis in Campania: brief overview of an Italian database

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12 December 2020

Premorbid body mass index and risk of amyotrophic lateral sclerosis

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12 December 2020

Caprylic triglyceride as a novel therapeutic approach to effectively improve the performance and attenuate the symptoms due to the motor neuron loss in ALS disease

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12 December 2020

Vitamin E serum levels and controlled supplementation and risk of amyotrophic lateral sclerosis

1 reference

12 December 2020

ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43.

1 reference

12 December 2020

Tat-activating regulatory DNA-binding protein regulates glycolysis in hepatocellular carcinoma by regulating the platelet isoform of phosphofructokinase through microRNA 520.

1 reference

12 December 2020

Prediagnostic body fat and risk of death from amyotrophic lateral sclerosis: the EPIC cohort

1 reference

12 December 2020

Lifetime physical activity and the risk of amyotrophic lateral sclerosis

1 reference

12 December 2020

Randomized double-blind placebo-controlled trial of acetyl-L-carnitine for ALS.

1 reference

12 December 2020

Body mass index (BMI) as predictor of ALSFRS-R score decline in ALS patients

1 reference

12 December 2020

Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice

1 reference

12 December 2020

Plasma progranulin concentrations are increased in patients with type 2 diabetes and obesity and correlated with insulin resistance

1 reference

12 December 2020

Hypolipidemia in patients with amyotrophic lateral sclerosis: a possible gender difference?

1 reference

12 December 2020

Dietary intake of fruits and beta-carotene is negatively associated with amyotrophic lateral sclerosis risk in Koreans: a case-control study.

1 reference

12 December 2020

Therapeutic exercise for people with amyotrophic lateral sclerosis or motor neuron disease

1 reference

12 December 2020

Metabolic signatures of amyotrophic lateral sclerosis reveal insights into disease pathogenesis

1 reference

12 December 2020

Vitamin D deficiency and its supplementation in patients with amyotrophic lateral sclerosis

1 reference

12 December 2020

Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis

1 reference

12 December 2020

High-caloric food supplements in the treatment of amyotrophic lateral sclerosis: A prospective interventional study

1 reference

12 December 2020

Autoimmune disease preceding amyotrophic lateral sclerosis: an epidemiologic study

1 reference

12 December 2020

TDP-43, an ALS linked protein, regulates fat deposition and glucose homeostasis

1 reference

12 December 2020

Progranulin: at the interface of neurodegenerative and metabolic diseases

1 reference

12 December 2020

Trials of antidiabetic drugs in amyotrophic lateral sclerosis: proceed with caution?

1 reference

12 December 2020

Vitamin D confers protection to motoneurons and is a prognostic factor of amyotrophic lateral sclerosis

1 reference

12 December 2020

Diabetes and risk of Parkinson's disease: an updated meta-analysis of case-control studies

1 reference

12 December 2020

Mitochondrial dysfunction and decrease in body weight of a transgenic knock-in mouse model for TDP-43.

1 reference

12 December 2020

TREM2 variant p.R47H as a risk factor for sporadic amyotrophic lateral sclerosis

1 reference

12 December 2020

Hypercaloric enteral nutrition in patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled phase 2 trial

1 reference

12 December 2020

A plural role for lipids in motor neuron diseases: energy, signaling and structure

1 reference

12 December 2020

Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis.

1 reference

12 December 2020

Increased amino acids levels and the risk of developing of hypertriglyceridemia in a 7-year follow-up

1 reference

12 December 2020

Physical activity and amyotrophic lateral sclerosis: a European population-based case-control study

1 reference

12 December 2020

AMPK activation: a therapeutic target for type 2 diabetes?

1 reference

12 December 2020

Dietary ω-3 polyunsaturated fatty acid intake and risk for amyotrophic lateral sclerosis

1 reference

12 December 2020

Separate responses of karyopherins to glucose and amino acid availability regulate nucleocytoplasmic transport

1 reference

12 December 2020

Metabolic therapy with Deanna Protocol supplementation delays disease progression and extends survival in amyotrophic lateral sclerosis (ALS) mouse model

1 reference

12 December 2020

FUS-regulated RNA metabolism and DNA damage repair: Implications for amyotrophic lateral sclerosis and frontotemporal dementia pathogenesis

1 reference

12 December 2020

Triggering receptor expressed on myeloid cells 2 (TREM2) promotes adipogenesis and diet-induced obesity.

1 reference

12 December 2020

Interaction of amyotrophic lateral sclerosis/frontotemporal lobar degeneration-associated fused-in-sarcoma with proteins involved in metabolic and protein degradation pathways

1 reference

12 December 2020

Activation of AMP-activated protein kinase α1 mediates mislocalization of TDP-43 in amyotrophic lateral sclerosis.

1 reference

12 December 2020

Systemic metabolism in frontotemporal dementia

1 reference

12 December 2020

Emerging concepts in Alzheimer's disease

1 reference

12 December 2020

Reversible behavioral phenotypes in a conditional mouse model of TDP-43 proteinopathies

1 reference

12 December 2020

Diabetes mellitus in amyotrophic lateral sclerosis: Dr. Jekyll or Mr. Hyde?

1 reference

12 December 2020

Association between diabetes and amyotrophic lateral sclerosis in Sweden

1 reference

12 December 2020

PGRN induces impaired insulin sensitivity and defective autophagy in hepatic insulin resistance

1 reference

12 December 2020

Antecedent Disease is Less Prevalent in Amyotrophic Lateral Sclerosis

1 reference

12 December 2020

A metabolic switch toward lipid use in glycolytic muscle is an early pathologic event in a mouse model of amyotrophic lateral sclerosis

1 reference

12 December 2020

Pre-morbid type 2 diabetes mellitus is not a prognostic factor in amyotrophic lateral sclerosis

1 reference

12 December 2020

Inactivation of EWS reduces PGC-1α protein stability and mitochondrial homeostasis

1 reference

12 December 2020

Risk of Amyotrophic Lateral Sclerosis in Patients With Diabetes: A Nationwide Population-Based Cohort Study

1 reference

12 December 2020

C9ORF72 GGGGCC Expanded Repeats Produce Splicing Dysregulation which Correlates with Disease Severity in Amyotrophic Lateral Sclerosis

1 reference

12 December 2020

Administration of progranulin (PGRN) triggers ER stress and impairs insulin sensitivity via PERK-eIF2α-dependent manner

1 reference

12 December 2020

Glycosphingolipids are modulators of disease pathogenesis in amyotrophic lateral sclerosis

1 reference

12 December 2020

Vitamin D is Not a Protective Factor in ALS.

1 reference

12 December 2020

The serum lipid profiles of amyotrophic lateral sclerosis patients: A study from south-west China and a meta-analysis

1 reference

12 December 2020

Plasma Free Amino Acid Profiles Predict Four-Year Risk of Developing Diabetes, Metabolic Syndrome, Dyslipidemia, and Hypertension in Japanese Population

1 reference

12 December 2020

Effect of lipid profile on prognosis in the patients with amyotrophic lateral sclerosis: Insights from the olesoxime clinical trial.

1 reference

12 December 2020

Motor neuron disease: Are diabetes and amyotrophic lateral sclerosis related?

1 reference

12 December 2020

Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions

1 reference

12 December 2020

FUS Interacts with HSP60 to Promote Mitochondrial Damage

1 reference

12 December 2020

Progranulin induces adipose insulin resistance and autophagic imbalance via TNFR1 in mice

1 reference

12 December 2020

Amyotrophic lateral sclerosis and denervation alter sphingolipids and up-regulate glucosylceramide synthase

1 reference

12 December 2020

Prognostic Factors in Amyotrophic Lateral Sclerosis: A Population-Based Study

1 reference

12 December 2020

Branched-chain and aromatic amino acid profiles and diabetes risk in Chinese populations

1 reference

12 December 2020

Physical activity and risk of Amyotrophic Lateral Sclerosis in a prospective cohort study

1 reference

12 December 2020

Antecedent Disease and Amyotrophic Lateral Sclerosis: What Is Protecting Whom?

1 reference

12 December 2020

Maple Syrup Decreases TDP-43 Proteotoxicity in a Caenorhabditis elegans Model of Amyotrophic Lateral Sclerosis (ALS).

1 reference

12 December 2020

C9orf72 BAC Mouse Model with Motor Deficits and Neurodegenerative Features of ALS/FTD.

1 reference

12 December 2020

Trehalose upregulates progranulin expression in human and mouse models of GRN haploinsufficiency: a novel therapeutic lead to treat frontotemporal dementia

1 reference

12 December 2020

The inhibition of TDP-43 mitochondrial localization blocks its neuronal toxicity

1 reference

12 December 2020

Distinct and shared functions of ALS-associated proteins TDP-43, FUS and TAF15 revealed by multisystem analyses.

1 reference

12 December 2020

ALS/FTD-associated FUS activates GSK-3β to disrupt the VAPB-PTPIP51 interaction and ER-mitochondria associations.

1 reference

12 December 2020

A high-fat jelly diet restores bioenergetic balance and extends lifespan in the presence of motor dysfunction and lumbar spinal cord motor neuron loss in TDP-43A315T mutant C57BL6/J mice.

1 reference

12 December 2020

Role of metabolism in neurodegenerative disorders.

1 reference

12 December 2020

Triheptanoin Protects Motor Neurons and Delays the Onset of Motor Symptoms in a Mouse Model of Amyotrophic Lateral Sclerosis

1 reference

12 December 2020

Omics to Explore Amyotrophic Lateral Sclerosis Evolution: the Central Role of Arginine and Proline Metabolism.

1 reference

12 December 2020

Assessment of CYP1A2 enzyme activity in relation to type-2 diabetes and habitual caffeine intake.

1 reference

12 December 2020

Association Between Dietary Intake and Function in Amyotrophic Lateral Sclerosis

1 reference

12 December 2020

Defective cholesterol metabolism in amyotrophic lateral sclerosis

1 reference

12 December 2020

Decoding ALS: from genes to mechanism.

1 reference

12 December 2020

Assessment and nutrition education in patients with amyotrophic lateral sclerosis

1 reference

12 December 2020

Early Cognitive/Social Deficits and Late Motor Phenotype in Conditional Wild-Type TDP-43 Transgenic Mice

1 reference

12 December 2020

TREM2 Promotes Microglial Survival by Activating Wnt/β-Catenin Pathway

1 reference

12 December 2020

Body mass index and amyotrophic lateral sclerosis: a study of US military veterans

1 reference

12 December 2020

TDP-43 mutations causing amyotrophic lateral sclerosis are associated with altered expression of RNA-binding protein hnRNP K and affect the Nrf2 antioxidant pathway.

1 reference

12 December 2020

Using induced pluripotent stem cells derived neurons to model brain diseases

1 reference

12 December 2020

Low IDL-B and high LDL-1 subfraction levels in serum of ALS patients

1 reference

12 December 2020

The role of pre-morbid diabetes on developing amyotrophic lateral sclerosis

1 reference

12 December 2020

Blood hemoglobin A1c levels and amyotrophic lateral sclerosis survival

1 reference

12 December 2020

Controlled placement of multiple CNS cell populations to create complex neuronal cultures.

1 reference

12 December 2020

Lipidomics Reveals Cerebrospinal-Fluid Signatures of ALS.

1 reference

12 December 2020

Association of Serum Retinol-Binding Protein 4 Concentration With Risk for and Prognosis of Amyotrophic Lateral Sclerosis

1 reference

12 December 2020

Lipidomics Analysis of Behavioral Variant Frontotemporal Dementia: A Scope for Biomarker Development.

1 reference

12 December 2020

Role of carnitine and its derivatives in the development and management of type 2 diabetes.

1 reference

12 December 2020

Multicentre, cross-cultural, population-based, case-control study of physical activity as risk factor for amyotrophic lateral sclerosis.

1 reference

12 December 2020

TDP-43 interacts with mitochondrial proteins critical for mitophagy and mitochondrial dynamics

1 reference

12 December 2020

Reduced Autophagy by a microRNA-mediated Signaling Cascade in Diabetes-induced Renal Glomerular Hypertrophy.

1 reference

12 December 2020

Differential toxicity of TAR DNA-binding protein 43 isoforms depends on their submitochondrial localization in neuronal cells

1 reference

12 December 2020

Medium-Chain Fatty Acids, Beta-Hydroxybutyric Acid and Genetic Modulation of the Carnitine Shuttle Are Protective in a Drosophila Model of ALS Based on TDP-43.

1 reference

12 December 2020

10.1186/S13024-018-0294-0

Identifiers

DOI

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:30509290%20AND%20SRC:MED&resulttype=core&format=json

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12 April 2020

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9 September 2019

https://scigraph.springernature.com/pub.10.1186/s13024-018-0294-0

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:30509290%20AND%20SRC:MED&resulttype=core&format=json

12 April 2020

PubMed publication ID

30509290

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