Detalhe da pesquisa
1.
Comprehensive evaluation of human-derived anti-poly-GA antibodies in cellular and animal models of C9orf72 disease.
Proc Natl Acad Sci U S A
; 119(49): e2123487119, 2022 12 06.
Artigo
Inglês
| MEDLINE | ID: mdl-36454749
2.
Translational profiling identifies a cascade of damage initiated in motor neurons and spreading to glia in mutant SOD1-mediated ALS.
Proc Natl Acad Sci U S A
; 112(50): E6993-7002, 2015 Dec 15.
Artigo
Inglês
| MEDLINE | ID: mdl-26621731
3.
Misfolded SOD1 is not a primary component of sporadic ALS.
Acta Neuropathol
; 134(1): 97-111, 2017 07.
Artigo
Inglês
| MEDLINE | ID: mdl-28247063
4.
Mutant TDP-43 within motor neurons drives disease onset but not progression in amyotrophic lateral sclerosis.
Acta Neuropathol
; 133(6): 907-922, 2017 06.
Artigo
Inglês
| MEDLINE | ID: mdl-28357566
5.
C1q induction and global complement pathway activation do not contribute to ALS toxicity in mutant SOD1 mice.
Proc Natl Acad Sci U S A
; 110(46): E4385-92, 2013 Nov 12.
Artigo
Inglês
| MEDLINE | ID: mdl-24170856
6.
ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43.
Proc Natl Acad Sci U S A
; 110(8): E736-45, 2013 Feb 19.
Artigo
Inglês
| MEDLINE | ID: mdl-23382207
7.
Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration.
Proc Natl Acad Sci U S A
; 110(47): E4530-9, 2013 Nov 19.
Artigo
Inglês
| MEDLINE | ID: mdl-24170860
8.
Enhancing mitochondrial calcium buffering capacity reduces aggregation of misfolded SOD1 and motor neuron cell death without extending survival in mouse models of inherited amyotrophic lateral sclerosis.
J Neurosci
; 33(11): 4657-71, 2013 Mar 13.
Artigo
Inglês
| MEDLINE | ID: mdl-23486940
9.
Stathmin-2 loss leads to neurofilament-dependent axonal collapse driving motor and sensory denervation.
Nat Neurosci
; 27(1): 34-47, 2024 Jan.
Artigo
Inglês
| MEDLINE | ID: mdl-37996528
10.
Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice.
Proc Natl Acad Sci U S A
; 106(11): 4465-70, 2009 Mar 17.
Artigo
Inglês
| MEDLINE | ID: mdl-19251638
11.
Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice.
Proc Natl Acad Sci U S A
; 105(21): 7594-9, 2008 May 27.
Artigo
Inglês
| MEDLINE | ID: mdl-18492803
12.
Therapeutically viable generation of neurons with antisense oligonucleotide suppression of PTB.
Nat Neurosci
; 24(8): 1089-1099, 2021 08.
Artigo
Inglês
| MEDLINE | ID: mdl-34083786
13.
Wild-type FUS corrects ALS-like disease induced by cytoplasmic mutant FUS through autoregulation.
Mol Neurodegener
; 16(1): 61, 2021 09 06.
Artigo
Inglês
| MEDLINE | ID: mdl-34488813
14.
Reduced C9ORF72 function exacerbates gain of toxicity from ALS/FTD-causing repeat expansion in C9orf72.
Nat Neurosci
; 23(5): 615-624, 2020 05.
Artigo
Inglês
| MEDLINE | ID: mdl-32284607
15.
Spinal subpial delivery of AAV9 enables widespread gene silencing and blocks motoneuron degeneration in ALS.
Nat Med
; 26(1): 118-130, 2020 01.
Artigo
Inglês
| MEDLINE | ID: mdl-31873312
16.
Antisense oligonucleotide therapy for neurodegenerative disease.
J Clin Invest
; 116(8): 2290-6, 2006 Aug.
Artigo
Inglês
| MEDLINE | ID: mdl-16878173
17.
An endogenous peptide marker differentiates SOD1 stability and facilitates pharmacodynamic monitoring in SOD1 amyotrophic lateral sclerosis.
JCI Insight
; 4(10)2019 05 16.
Artigo
Inglês
| MEDLINE | ID: mdl-31092730
18.
Overriding FUS autoregulation in mice triggers gain-of-toxic dysfunctions in RNA metabolism and autophagy-lysosome axis.
Elife
; 82019 02 12.
Artigo
Inglês
| MEDLINE | ID: mdl-30747709
19.
ALS/FTD-Linked Mutation in FUS Suppresses Intra-axonal Protein Synthesis and Drives Disease Without Nuclear Loss-of-Function of FUS.
Neuron
; 100(4): 816-830.e7, 2018 11 21.
Artigo
Inglês
| MEDLINE | ID: mdl-30344044
20.
Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs.
Neuron
; 90(3): 535-50, 2016 05 04.
Artigo
Inglês
| MEDLINE | ID: mdl-27112497