Lymphoblastoid cell lines as a model to understand amyotrophic lateral sclerosis disease mechanisms

scientific article

Lymphoblastoid cell lines as a model to understand amyotrophic lateral sclerosis disease mechanisms is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1242/DMM.031625
P932PMC publication ID5897724
P698PubMed publication ID29419416

P50authorMatteo BordoniQ58952002
Lorenzo DrufucaQ95990317
Cristina CeredaQ37842077
P2093author name stringStella Gagliardi
Mauro Ceroni
Orietta Pansarasa
Luca Diamanti
Stefano Bernuzzi
Sabrina La Salvia
Daisy Sproviero
Rosa Trotti
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Effect of nitric oxide on lymphocytes from sporadic amyotrophic lateral sclerosis patients: toxic or protective role?Q28275312
SOD1 mRNA expression in sporadic amyotrophic lateral sclerosisQ28279971
FUS Interacts with HSP60 to Promote Mitochondrial DamageQ28547727
Enhancing Mitofusin/Marf ameliorates neuromuscular dysfunction in Drosophila models of TDP-43 proteinopathies.Q50784802
Altered age-related changes in bioenergetic properties and mitochondrial morphology in fibroblasts from sporadic amyotrophic lateral sclerosis patients.Q51597460
Skeletal Muscle Is a Primary Target of SOD1G93A-Mediated ToxicityQ62397259
Morphological abnormalities in mitochondria of the skin of patients with sporadic amyotrophic lateral sclerosisQ83178534
TDP-43 and FUS: a nuclear affairQ84433614
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El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosisQ29619074
Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse modelsQ30572264
Focal degeneration of astrocytes in amyotrophic lateral sclerosis.Q33350786
In vivo accumulation of Helicobacter pylori products, NOD1, ubiquitinated proteins and proteasome in a novel cytoplasmic structureQ33543097
Mitochondrial dysfunction and intracellular calcium dysregulation in ALSQ34103782
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Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic miceQ34664325
Altered intracellular localization of SOD1 in leukocytes from patients with sporadic amyotrophic lateral sclerosisQ35025204
An over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1Q35882558
Early and gender-specific differences in spinal cord mitochondrial function and oxidative stress markers in a mouse model of ALSQ35891904
TDP-43 and FUS en route from the nucleus to the cytoplasmQ36336278
Nonnative SOD1 trimer is toxic to motor neurons in a model of amyotrophic lateral sclerosisQ36498352
The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neuronsQ37286990
Mitochondria-associated membrane collapse is a common pathomechanism in SIGMAR1- and SOD1-linked ALSQ37514803
Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyondQ37642283
Protein misdirection inside and outside motor neurons in Amyotrophic Lateral Sclerosis (ALS): a possible clue for therapeutic strategiesQ37954610
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The changing scene of amyotrophic lateral sclerosisQ38086771
Role of protein misfolding and proteostasis deficiency in protein misfolding diseases and agingQ38172142
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Disturbed mitochondrial dynamics and neurodegenerative disordersQ38285228
Misfolded SOD1 Accumulation and Mitochondrial Association Contribute to the Selective Vulnerability of Motor Neurons in Familial ALS: Correlation to Human DiseaseQ38675517
Gene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts.Q38772926
Mutant SOD1G93A triggers mitochondrial fragmentation in spinal cord motor neurons: neuroprotection by SIRT3 and PGC-1α.Q38844786
Overexpression of nuclear FUS induces neuronal cell deathQ38929777
Decoding ALS: from genes to mechanism.Q39004348
Metabolic Dysfunctions in Amyotrophic Lateral Sclerosis Pathogenesis and Potential Metabolic TreatmentsQ39101800
TDP-43 and Cytoskeletal Proteins in ALS.Q39279950
The role of mitochondria in amyotrophic lateral sclerosisQ39409879
The ALS-linked E102Q mutation in Sigma receptor-1 leads to ER stress-mediated defects in protein homeostasis and dysregulation of RNA-binding proteins.Q41636922
An EBV-transformed owl monkey B-lymphocyte cell lineQ42814431
In vitro studies in VCP-associated multisystem proteinopathy suggest altered mitochondrial bioenergeticsQ46148248
Lon protease: a novel mitochondrial matrix protein in the interconnection between drug-induced mitochondrial dysfunction and endoplasmic reticulum stress.Q47757120
P275copyright licenseCreative Commons Attribution 3.0 UnportedQ14947546
P6216copyright statuscopyrightedQ50423863
P4510describes a project that usesImageJQ1659584
P433issue3
P921main subjectamyotrophic lateral sclerosisQ206901
P577publication date2018-03-26
P1433published inDisease Models & MechanismsQ1524006
P1476titleLymphoblastoid cell lines as a model to understand amyotrophic lateral sclerosis disease mechanisms
P478volume11

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cites work (P2860)
Q64072993Extensive epigenetic and transcriptomic variability between genetically identical human B-lymphoblastoid cells with implications in pharmacogenomics research
Q96304785In vitro Models of Neurodegenerative Diseases
Q98224396Mitochondrial Dysfunction, Neurogenesis, and Epigenetics: Putative Implications for Amyotrophic Lateral Sclerosis Neurodegeneration and Treatment
Q92246835Nuclear Phospho-SOD1 Protects DNA from Oxidative Stress Damage in Amyotrophic Lateral Sclerosis
Q60529524Pathological Proteins Are Transported by Extracellular Vesicles of Sporadic Amyotrophic Lateral Sclerosis Patients
Q64912499Proteostasis and ALS: protocol for a phase II, randomised, double-blind, placebo-controlled, multicentre clinical trial for colchicine in ALS (Co-ALS).
Q61799556RNA-Seq profiling in peripheral blood mononuclear cells of amyotrophic lateral sclerosis patients and controls
Q99614266Replication Stress Induces Global Chromosome Breakage in the Fragile X Genome
Q92320484The relationship between glutathione levels in leukocytes and ocular clinical parameters in glaucoma

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