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Impaired Autophagy and Defective Mitochondrial Function: Converging Paths on the Road to Motor Neuron DegenerationA comprehensive review of amyotrophic lateral sclerosisMicroglia centered pathogenesis in ALS: insights in cell interconnectivityRedox regulation in amyotrophic lateral sclerosisThe anatomical distribution of genetic associations.PPARĪ³ as a therapeutic target to rescue mitochondrial function in neurological diseaseThe Cu, Zn Superoxide Dismutase: Not Only a Dismutase EnzymeThe complex molecular biology of amyotrophic lateral sclerosis (ALS)Whole transcriptome analysis of a reversible neurodegenerative process in Drosophila reveals potential neuroprotective genes.Protein aggregation and protein instability govern familial amyotrophic lateral sclerosis patient survival.Aggresome-forming TTRAP mediates pro-apoptotic properties of Parkinson's disease-associated DJ-1 missense mutations.Nuclear export of misfolded SOD1 mediated by a normally buried NES-like sequence reduces proteotoxicity in the nucleus.The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solutionParkinson's disease DJ-1 L166P alters rRNA biogenesis by exclusion of TTRAP from the nucleolus and sequestration into cytoplasmic aggregates via TRAF6Synergic prodegradative activity of Bicalutamide and trehalose on the mutant androgen receptor responsible for spinal and bulbar muscular atrophy.Bee venom effects on ubiquitin proteasome system in hSOD1(G85R)-expressing NSC34 motor neuron cellsAltered intracellular localization of SOD1 in leukocytes from patients with sporadic amyotrophic lateral sclerosis.The anabolic/androgenic steroid nandrolone exacerbates gene expression modifications induced by mutant SOD1 in muscles of mice models of amyotrophic lateral sclerosis.Time-Point Dependent Activation of Autophagy and the UPS in SOD1G93A Mice Skeletal MuscleNeuroprotective Effect of Bexarotene in the SOD1(G93A) Mouse Model of Amyotrophic Lateral Sclerosis.Transcriptional induction of the heat shock protein B8 mediates the clearance of misfolded proteins responsible for motor neuron diseases.Clearance of the mutant androgen receptor in motoneuronal models of spinal and bulbar muscular atrophy.FTD and ALS: a tale of two diseasesDifferential autophagy power in the spinal cord and muscle of transgenic ALS mice.Overexpression of survival motor neuron improves neuromuscular function and motor neuron survival in mutant SOD1 mice.The chaperone HSPB8 reduces the accumulation of truncated TDP-43 species in cells and protects against TDP-43-mediated toxicity.Dysregulation of axonal transport and motorneuron diseases.Amyotrophic lateral sclerosis and skeletal muscle: an update.DNA strand breaks and TDP-43 mislocation are absent in the murine hSOD1G93A model of amyotrophic lateral sclerosis in vivo and in vitro.The small heat shock protein B8 (HSPB8) efficiently removes aggregating species of dipeptides produced in C9ORF72-related neurodegenerative diseases.Inhibition of retrograde transport modulates misfolded protein accumulation and clearance in motoneuron diseases.Blood trace metals in a sporadic amyotrophic lateral sclerosis geographical cluster.Muscle cells and motoneurons differentially remove mutant SOD1 causing familial amyotrophic lateral sclerosisChronic oxidative damage together with genome repair deficiency in the neurons is a double whammy for neurodegeneration: Is damage response signaling a potential therapeutic target?A differential autophagy-dependent response to DNA double-strand breaks in bone marrow mesenchymal stem cells from sporadic ALS patients.Parkin Protects Against Misfolded SOD1 Toxicity by Promoting Its Aggresome Formation and Autophagic Clearance.Impairment of SOD1-G93A motility is linked to mitochondrial movement in axons of hippocampal neurons.Mitochondrial DNA damage and reactive oxygen species in neurodegenerative disease.Modeling Neurovascular Disorders and Therapeutic Outcomes with Human-Induced Pluripotent Stem Cells.DNA plasticity and damage in amyotrophic lateral sclerosis.
P2860
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P2860
description
2007 nĆ® lÅ«n-bĆ»n
@nan
2007 Õ©ÕøÖÕ”ÕÆÕ”Õ¶Õ« Õ
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@hyw
2007 Õ©Õ¾Õ”ÕÆÕ”Õ¶Õ« Õ°ÕøÖÕ¬Õ«Õ½Õ«Õ¶ Õ°ÖÕ”ÕæÕ”ÖÕ”ÕÆÕ¾Õ”Õ® Õ£Õ«ÕæÕ”ÕÆÕ”Õ¶ Õ°ÕøÕ¤Õ¾Õ”Õ®
@hy
2007幓ć®č«ę
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2007幓č«ę
@yue
2007幓č«ę
@zh-hant
2007幓č«ę
@zh-hk
2007幓č«ę
@zh-mo
2007幓č«ę
@zh-tw
2007幓č®ŗę
@wuu
name
Mutation of SOD1 in ALS: a gain of a loss of function
@ast
Mutation of SOD1 in ALS: a gain of a loss of function
@en
Mutation of SOD1 in ALS: a gain of a loss of function
@en-gb
Mutation of SOD1 in ALS: a gain of a loss of function
@nl
type
label
Mutation of SOD1 in ALS: a gain of a loss of function
@ast
Mutation of SOD1 in ALS: a gain of a loss of function
@en
Mutation of SOD1 in ALS: a gain of a loss of function
@en-gb
Mutation of SOD1 in ALS: a gain of a loss of function
@nl
prefLabel
Mutation of SOD1 in ALS: a gain of a loss of function
@ast
Mutation of SOD1 in ALS: a gain of a loss of function
@en
Mutation of SOD1 in ALS: a gain of a loss of function
@en-gb
Mutation of SOD1 in ALS: a gain of a loss of function
@nl
P2093
P2860
P50
P3181
P356
P1476
Mutation of SOD1 in ALS: a gain of a loss of function
@en
P2093
Daniela Sau
Elena Bolzoni
Elisa Onesto
Isabella Palazzolo
Laura Vitellaro-Zuccarello
Serena Guarnieri
Silvia De Biasi
Silvia Simeoni
P2860
P304
P3181
P356
10.1093/HMG/DDM110
P407
P577
2007-07-01T00:00:00Z