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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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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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Patterns of synaptic loss in human amyotrophic lateral sclerosis spinal cord: a clinicopathological study.

• Authors : Aousji, Oumayma; Feldengut, Simone; Braak, Heiko

Subjects: info:eu-repo/classification/ddc/610; Humans; MiceDE

• Source: Acta Neuropathologica Communications 11(1), 120 (2023). doi:10.1186/s40478-023-01616-8

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Modelling TDP-43 proteinopathy in Drosophila uncovers shared and neuron-specific targets across ALS and FTD relevant circuits

• Authors : Godfrey, R Keating; Alsop, Eric; Bjork, Reed T

Subjects: ALS; Drosophila; FTD

• Source: Translational Neuroscience

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Subpial delivery of adeno-associated virus 9-synapsin-caveolin-1 ( AAV9-SynCav1) preserves motor neuron and neuromuscular junction morphology, motor function, delays disease onset, and extends survival in hSOD1G93A mice

• Authors : Shanshan Wang; Taiga Ichinomiya; Paul Savchenko

Subjects: Amyotrophic Lateral Sclerosis* / genetics; Amyotrophic Lateral Sclerosis* / therapy; Animals

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Astrocyte-targeting RNA interference against mutated superoxide dismutase 1 induces motoneuron plasticity and protects fast-fatigable motor units in a mouse model of amyotrophic lateral sclerosis.

• Authors : Rochat, C.; Bernard-Marissal, N.; Källstig, E.

Subjects: Amyotrophic Lateral Sclerosis/genetics; Amyotrophic Lateral Sclerosis/metabolism; Amyotrophic Lateral Sclerosis/therapy

• Source: Glia, vol. 70, no. 5, pp. 842-857

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Motor neurons use push-pull signals to direct vascular remodeling critical for their connectivity

• Authors : Martins, Luis; Brambilla, Ilaria; Motta, Alessia

Subjects: VEGF; Axon guidance; Cell signaling

• Source: ISSN: 0896-6273 ; Neuron, vol. 110, no. 24 (2022) p. 4090-4107.e11.

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UXT chaperone prevents proteotoxicity by acting as an autophagy adaptor for p62-dependent aggrephagy

• Authors : Min Ji Yoon; Boyoon Choi; Eun Jin Kim

Subjects: Animals; Autophagy / drug effects; Autophagy / genetics

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