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ATAXIN-2 intermediate-length polyglutamine expansions elicit ALS-associated metabolic and immune phenotypes

• Authors : Vieira de Sá, Renata; Sudria-Lopez, Emma; Cañizares Luna, Marta ...

Subjects: Amyotrophic Lateral Sclerosis/genetics; Animals; Ataxin-2/genetics

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ALS-associated C21ORF2 variant disrupts DNA damage repair, mitochondrial metabolism, neuronal excitability and NEK1 levels in human motor neurons

• Authors : Zelina, Pavol; de Ruiter, Anna Aster; Kolsteeg, Christy ...

Subjects: Amyotrophic Lateral Sclerosis/genetics; Animals; DNA Damage

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Stabilization of V1 interneuron-motor neuron connectivity ameliorates motor phenotype in a mouse model of ALS

• Authors : Mora, Santiago; Stuckert, Anna; von Huth Friis, Rasmus

Subjects: Animals; Amyotrophic Lateral Sclerosis/genetics; Interneurons/metabolism

• Source: Mora , S , Stuckert , A , von Huth Friis , R , Pietersz , K , Noes-Holt , G , Montañana-Rosell , R , Wang , H , Sørensen , A T , Selvan , R , Verhaagen , J & Allodi , I 2024 , ' Stabilization of V1

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Nucleolar reorganization after cellular stress is orchestrated by SMN shuttling between nuclear compartments.

• Authors : Musawi, Shaqraa; Donnio, Lise-Marie; Zhao, Zehui

Subjects: RNA-Binding Proteins; Nerve Tissue Proteins; SMN Complex Proteins

• Source: Nature Communications, 14 (1), 7384 (2023-11-15)

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SorCS2 binds progranulin to regulate motor neuron development

• Authors : Thomasen, Pernille Bogetofte; Salasova, Alena; Kjaer-Sorensen, Kasper

Subjects: CP: Developmental biology; CP: Neuroscience; SorCS2

• Source: Thomasen , P B , Salasova , A , Kjaer-Sorensen , K , Woloszczuková , L , Lavický , J , Login , H , Tranberg-Jensen , J , Almeida , S , Beel , S , Kavková , M , Qvist , P , Kjolby , M , Ovesen , P L

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FUS-ALS hiPSC-derived astrocytes impair human motor units through both gain-of-toxicity and loss-of-support mechanisms

• Authors : Stoklund Dittlau, Katarina; Terrie, Lisanne; Baatsen, Pieter

Subjects: Amyotrophic lateral sclerosis; Astrocyte; Cytokines

• Source: Stoklund Dittlau , K , Terrie , L , Baatsen , P , Kerstens , A , De Swert , L , Janky , R , Corthout , N , Masrori , P , Van Damme , P , Hyttel , P , Meyer , M , Thorrez , L , Freude , K & Van Den

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FUS ALS neurons activate major stress pathways and reduce translation as an early protective mechanism against neurodegeneration.

• Authors : Szewczyk, Barbara; Günther, René; Japtok, Julia

Subjects: info:eu-repo/classification/ddc/610; Humans; Amyotrophic Lateral Sclerosis: pathologyDE

• Source: Cell reports 42(2), 112025 (2023). doi:10.1016/j.celrep.2023.112025

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Integrative proteomics highlight presynaptic alterations and c-Jun misactivation as convergent pathomechanisms in ALS

• Authors : Aly, Amr; Laszlo, Zsofia I.; Barletta, Francesca

Subjects: info:eu-repo/classification/ddc/610; Humans; Amyotrophic Lateral Sclerosis: pathologyDE

• Source: Acta neuropathologica -, - (2023). doi:10.1007/s00401-023-02611-y

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Multiomics and machine-learning identify novel transcriptional and mutational signatures in amyotrophic lateral sclerosis.

• Authors : Catanese, Alberto; Rajkumar, Sandeep; Mulaw, Medhanie

Subjects: info:eu-repo/classification/ddc/610; Humans; Amyotrophic Lateral Sclerosis: metabolismDE

• Source: Brain 146(9), 3770 - 3782 (2023). doi:10.1093/brain/awad075

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Live Cell Imaging of ATP Levels Reveals Metabolic Compartmentalization within Motoneurons and Early Metabolic Changes in FUS ALS Motoneurons

• Authors : Zimyanin, Vitaly L.; Pielka, Anna-Maria; Andersen, Peter M.

Subjects: info:eu-repo/classification/ddc/570; Humans; Amyotrophic Lateral Sclerosis: metabolismDE

• Source: Cells 12(10), 1352 (2023). doi:10.3390/cells12101352 special issue: "Amyotrophic Lateral Sclerosis: From Molecular Mechanisms to Therapeutic Opportunities"

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