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ProteomicsDistinct amyloid fibril structures formed by ALS-causing SOD1 mutants G93A and D101N
We expressed and purified recombinant full-length wild-type human SOD1 and its G93A and D101N variants in Expi293F cells where the initiating Met was removed and the N-terminus at Ala1 was acetylated, and identified N-terminal acetylation of the mammalian cell–purified G93A , D101N , and wild-type S...
ORGANISM(S): Homo sapiens (Human) 
2025-10-20 | PXD066116 | Pride
Amyotrophic lateral sclerosis (als)  sod1 mutants  amyloid fibrils  cryo-em structure  toxicity. 
Proteomicssuperoxide dismutase 1 (SOD1) trimer interactome from mouse brain, spinal cord, and muscle tissues
In this work, we aimed to discover the protein binding partners of SOD1 trimers in brain, spinal cord, and skeletal muscle of mice tissue lysate.
ORGANISM(S): Mus musculus (Mouse) 
2025-10-06 | PXD050873 | Pride
Oligomers Interactome Sod1 
TranscriptomicsmiRNA expression in fast and slow skeletal muscle of wild type and SOD1-G93A mice
Amyotrophic lateral sclerosis (ALS) is a lethal motor neuron disease that progressively debilitates neuronal cells that control voluntary muscle activity. In a mouse model of ALS that expresses mutated human superoxide dismutase 1 (SOD1-G93A) skeletal muscle is one of the tissues affected early by m...
ORGANISM(S): Mus musculus 
2014-03-04 | E-GEOD-55507 | biostudies-arrayexpress
ProteomicsAmyotrophic Lateral Sclerosis-associated Mutants of SOD1 Modulate miRNA Biogenesis through Aberrant Interactions with Exportin 5
Mutations in SOD1 (Superoxide Dismutase 1) gene are associated with amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease. By employing ascorbate peroxidase (APEX)-based proximity labeling, coupled with LC-MS/MS analysis, we uncovered 37 and 28 proteins exhibiting higher abundance i...
ORGANISM(S): Homo sapiens (Human) Mus musculus (Mouse) 
2024-01-26 | PXD035452 | Pride
Lc-ms affinity purification sod1 apex 
TranscriptomicsTranscriptome Profiling following Neuronal and Glial Expression of ALS-linked SOD1 in Drosophila
Amyotrophic Lateral Sclerosis (ALS) is generally a late onset neurodegenerative disease. Mutations in the Cu/Zn superoxide dismutase 1 (SOD1) gene accounts for approximately 20% of familial ALS and 2% of all ALS cases. Although a number of hypothesis have been proposed to explain mutant SOD1 toxic...
ORGANISM(S): Drosophila melanogaster 
2012-06-30 | E-GEOD-37148 | biostudies-arrayexpress
TranscriptomicsProfiling wt SOD versus ALS SOD1(G93A) mutant
Whole-genome profiling of SH-SY5Y cells was done on neuroblastoma SH-SY5Y stably transfected with cDNAs coding for SOD1WT or the mutant SOD1(G93A) protein. Five wt SOD versus five mutant SOD
ORGANISM(S): Homo sapiens 
2010-12-22 | E-GEOD-21298 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of mouse motorneurons during progression of the disease in transgenic SOD1(G93A) mice that develop motorneuron loss
To identify candidate genes that may be involved in motoneuron degeneration, we combined laser capture microdissection with microarray technology. Gene expression in motoneurons was analyzed during the progression of the disease in transgenic SOD1(G93A) mice that develop motoneuron loss. Three major...
ORGANISM(S): Mus musculus 
2005-10-30 | E-MEXP-453 | biostudies-arrayexpress
TranscriptomicsTranscriptional profile of primary astrocytes expressing ALS-linked mutant SOD1.
Amyotrophic lateral sclerosis (ALS) is caused by the progressive degeneration of motor neurons. Mutations in the Cu/Zn superoxide dismutase (SOD1) are found in about 20% of patients with familial ALS. Mutant SOD1 causes motor neuron death through an acquired toxic property. Although, molecular mecha...
ORGANISM(S): Rattus norvegicus 
2008-12-18 | E-GEOD-7441 | biostudies-arrayexpress
ProteomicsAmyotrophic Lateral Sclerosis-Associated Mutants of SOD1 Perturb mRNA Splicing through Aberrant Interactions with SRSF2
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that impacts neurons in the brain and spinal cord. Although a substantial number of studies have been conducted, much remains to be learned about the cellular mechanisms underlying ALS. In this study, we employed an engineered ascor...
ORGANISM(S): Homo sapiens (Human) 
2025-06-16 | PXD050785 | Pride
Sod1 Als Affinity pulldown Lc-msms 
TranscriptomicsPathways Disrupted in Human ALS Motor Neurons Identified Through Genetic Correction of Mutant SOD1
Although many distinct mutations in a variety of genes are known to cause Amyotrophic Lateral Sclerosis (ALS), it remains poorly understood how they selectively impact motor neuron biology and whether they converge on common pathways to cause neural degeneration. Here, we have combined reprogramming...
ORGANISM(S): Homo sapiens 
2014-02-03 | E-GEOD-54409 | biostudies-arrayexpress
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