Detalhe da pesquisa
1.
Sphingolipids metabolism alteration in the central nervous system: Amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases.
Semin Cell Dev Biol
; 112: 82-91, 2021 04.
Artigo
Inglês
| MEDLINE | ID: mdl-33160824
2.
Atxn2-CAG100-KnockIn mouse spinal cord shows progressive TDP43 pathology associated with cholesterol biosynthesis suppression.
Neurobiol Dis
; 152: 105289, 2021 05.
Artigo
Inglês
| MEDLINE | ID: mdl-33577922
3.
Longitudinal transcriptomic analysis of altered pathways in a CHMP2Bintron5-based model of ALS-FTD.
Neurobiol Dis
; 136: 104710, 2020 03.
Artigo
Inglês
| MEDLINE | ID: mdl-31837425
4.
A transgenic mouse expressing CHMP2Bintron5 mutant in neurons develops histological and behavioural features of amyotrophic lateral sclerosis and frontotemporal dementia.
Hum Mol Genet
; 25(15): 3341-3360, 2016 08 01.
Artigo
Inglês
| MEDLINE | ID: mdl-27329763
5.
Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis.
Acta Neuropathol
; 133(6): 887-906, 2017 06.
Artigo
Inglês
| MEDLINE | ID: mdl-28243725
6.
APP-dependent glial cell line-derived neurotrophic factor gene expression drives neuromuscular junction formation.
FASEB J
; 30(5): 1696-711, 2016 05.
Artigo
Inglês
| MEDLINE | ID: mdl-26718890
7.
Evaluating Behavior in Mouse Models of the Behavioral Variant of Frontotemporal Dementia: Which Test for Which Symptom?
Neurodegener Dis
; 16(3-4): 127-39, 2016.
Artigo
Inglês
| MEDLINE | ID: mdl-26517704
8.
Investigating the contribution of VAPB/ALS8 loss of function in amyotrophic lateral sclerosis.
Hum Mol Genet
; 22(12): 2350-60, 2013 Jun 15.
Artigo
Inglês
| MEDLINE | ID: mdl-23446633
9.
Degeneration of serotonergic neurons in amyotrophic lateral sclerosis: a link to spasticity.
Brain
; 136(Pt 2): 483-93, 2013 Feb.
Artigo
Inglês
| MEDLINE | ID: mdl-23114367
10.
Cortical hyperexcitability in mouse models and patients with amyotrophic lateral sclerosis is linked to noradrenaline deficiency.
Sci Transl Med
; 16(738): eadg3665, 2024 Mar 13.
Artigo
Inglês
| MEDLINE | ID: mdl-38478631
11.
Dynein mutations associated with hereditary motor neuropathies impair mitochondrial morphology and function with age.
Neurobiol Dis
; 58: 220-30, 2013 Oct.
Artigo
Inglês
| MEDLINE | ID: mdl-23742762
12.
A mouse model of Schwartz-Jampel syndrome reveals myelinating Schwann cell dysfunction with persistent axonal depolarization in vitro and distal peripheral nerve hyperexcitability when perlecan is lacking.
Am J Pathol
; 180(5): 2040-55, 2012 May.
Artigo
Inglês
| MEDLINE | ID: mdl-22449950
13.
Activation of microglial N-methyl-D-aspartate receptors triggers inflammation and neuronal cell death in the developing and mature brain.
Ann Neurol
; 72(4): 536-49, 2012 Oct.
Artigo
Inglês
| MEDLINE | ID: mdl-23109148
14.
Mutations in cytoplasmic dynein lead to a Huntington's disease-like defect in energy metabolism of brown and white adipose tissues.
Biochim Biophys Acta
; 1812(1): 59-69, 2011 Jan.
Artigo
Inglês
| MEDLINE | ID: mdl-20887786
15.
A point mutation in the dynein heavy chain gene leads to striatal atrophy and compromises neurite outgrowth of striatal neurons.
Hum Mol Genet
; 19(22): 4385-98, 2010 Nov 15.
Artigo
Inglês
| MEDLINE | ID: mdl-20807776
16.
Alteration of the Neuromuscular Junction and Modifications of Muscle Metabolism in Response to Neuron-Restricted Expression of the CHMP2Bintron5 Mutant in a Mouse Model of ALS-FTD Syndrome.
Biomolecules
; 12(4)2022 03 24.
Artigo
Inglês
| MEDLINE | ID: mdl-35454086
17.
Extracellular Vesicles in Amyotrophic Lateral Sclerosis.
Life (Basel)
; 13(1)2022 Dec 31.
Artigo
Inglês
| MEDLINE | ID: mdl-36676070
18.
Repurposing of Trimetazidine for amyotrophic lateral sclerosis: A study in SOD1G93A mice.
Br J Pharmacol
; 179(8): 1732-1752, 2022 04.
Artigo
Inglês
| MEDLINE | ID: mdl-34783031
19.
Nogo receptor antagonizes p75NTR-dependent motor neuron death.
Proc Natl Acad Sci U S A
; 105(2): 740-5, 2008 Jan 15.
Artigo
Inglês
| MEDLINE | ID: mdl-18182498
20.
Skeletal Muscle Metabolism: Origin or Prognostic Factor for Amyotrophic Lateral Sclerosis (ALS) Development?
Cells
; 10(6)2021 06 09.
Artigo
Inglês
| MEDLINE | ID: mdl-34207859