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R-Roscovitine improves motoneuron function in mouse models for Spinal Muscular Atrophy.

ABSTRACT: R-Roscovitine improves motoneuron function in mouse models for Spinal Muscular Atrophy.

PROVIDER: PRJNA448923 | ENA |

REPOSITORIES: ENA

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			Action	DRS
	SRR6949930.fastq.gz	Fastqsanger.gz
	SRR6949931.fastq.gz	Fastqsanger.gz
	SRR6949932.fastq.gz	Fastqsanger.gz
	SRR6949933.fastq.gz	Fastqsanger.gz
	SRR6949934.fastq.gz	Fastqsanger.gz

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R-Roscovitine improves motoneuron function in mouse models for Spinal Muscular Atrophy.

Project description:Neurotransmission defects and motoneuron degeneration are hallmarks of Spinal Muscular Atrophy, a monogenetic disease caused by the deficiency of the SMN protein. In the present study, we show that systemic application of R-Roscovitine - a Cav2.1 / Cav2.2 channel modifier and a Cyclin-dependent kinase 5 (Cdk-5) inhibitor - significantly improved survival of SMA mice. In addition, R-Roscovitine increased Cav2.1 channel density and sizes of the motor endplates. In vitro, R-Roscovitine restored axon lengths and growth cone sizes of Smn-deficient motoneurons corresponding to enhanced spontaneous Ca2+ influx and elevated Cav2.2 channel cluster formations independent of its capability to inhibit Cdk-5. Acute application of R-Roscovitine at the neuromuscular junction significantly increased evoked neurotransmitter release, the frequency of spontaneous miniature potentials, and lowered the activation threshold of silent terminals. These data indicate that R-Roscovitine improves Ca2+ signaling and Ca2+ homeostasis in Smn-deficient motoneurons which is generally crucial for motoneuron differentiation, maturation, and function.

2020-01-28 | GSE112771 | GEO

Transcription profiling of muscular and neuronal mouse mutant: spinal muscular atrophy(SMA)

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Gene Expression Analysis of spinal cord obtained from a mouse model of severe Spinal Muscular Atrophy (SMA)

Project description:Spinal Muscular Atrophy (SMA) is an autosomal recessive motor neuron disease and is the second most common genetic disorder leading to death in childhood. No effective therapy is currently available. It has been suggested that β-lactam antibiotics such as ceftriaxone may offer neuroprotection in motoneuron disease. We investigated the therapeutic effect of ceftriaxone in a murine model of SMA. Microarray technology was used to assess the global gene expression profile of spinal cord obtained by ceftriaxone-treated and vehicle treated SMA mice.

2013-12-01 | GSE19674 | GEO

Gene Expression Analysis of spinal cord obtained from a mouse model of severe Spinal Muscular Atrophy (SMA)

Project description:Spinal Muscular Atrophy (SMA) is an autosomal recessive motor neuron disease and is the second most common genetic disorder leading to death in childhood. No effective therapy is currently available. It has been suggested that M-NM-2-lactam antibiotics such as ceftriaxone may offer neuroprotection in motoneuron disease. We investigated the therapeutic effect of ceftriaxone in a murine model of SMA. Microarray technology was used to assess the global gene expression profile of spinal cord obtained by ceftriaxone-treated and vehicle treated SMA mice. Comparative Gene Expression Analysis. The microarray data derived from three different groups: wildtype controls, transgenic SMA (vehicle treated) and ceftriaxone-treated SMA mice. Each population consists of four RNA profiling samples.

2013-12-01 | E-GEOD-19674 | biostudies-arrayexpress

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Transcription profiling of mouse spinal chord from a mouse model of severe spinal muscular atrophy

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Cell-mediated cytotoxicity surrounding the brain in spinal muscular atrophy

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Skeletal muscle mitochondrial dysfunction in spinal muscular atrophy

Project description:Skeletal muscle mitochondrial dysfunction in spinal muscular atrophy

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Increased Systemic HSP70B Levels in Spinal Muscular Atrophy Infants

Project description:Increased Systemic HSP70B Levels in Spinal Muscular Atrophy Infants

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Pathologic alteration in the proteome of synaptosomes from the mouse model of spinal muscular atrophy

Project description:In this study, label-free quantitative proteomic analysis was performed using the Smn 2B/- mouse model to identify and investigate significant changes in protein abundance that may be related to the pathogenesis and neurodegeneration oberved in spinal muscular atrophy (SMA)

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