Browse
Submit Data
Databases
API
Help

Dataset Information

0 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Region-specific pathogenesis and dissipation of intraregional heterogeneity in cerebellar neurodegeneration

ABSTRACT: Region-specific pathogenesis and dissipation of intraregional heterogeneity in cerebellar neurodegeneration

PROVIDER: PRJNA1075799 | ENA |

REPOSITORIES: ENA

ACCESS DATA

Json Xml

Dataset's files

Source:

			Action	DRS
	SRR27950600_1.fastq.gz	Fastqsanger.gz
	SRR27950600_2.fastq.gz	Fastqsanger.gz
	SRR27950601_1.fastq.gz	Fastqsanger.gz
	SRR27950601_2.fastq.gz	Fastqsanger.gz
	SRR27950602_1.fastq.gz	Fastqsanger.gz

Items per page:

1 - 5 of 98

Similar Datasets

Region-specific pathogenesis and dissipation of intraregional heterogeneity in cerebellar neurodegeneration

Project description:Heterogeneity and selective vulnerability are among the key features of the healthy and diseased brain. Cerebellum contains majority of brain cells, and is thought to be relatively uniform in structure. We explore heterogeneity in the context of healthy cerebellum and during selective vulnerability in cerebellar disease.

2024-05-16 | GSE255633 | GEO

Longitudinal single-cell transcriptional dynamics throughout neurodegeneration in SCA1

Project description:Neurodegeneration is a protracted process involving progressive changes in myriad cell types that ultimately result in the death of vulnerable neuronal populations. To dissect how individual cell types within a heterogeneous tissue contribute to the pathogenesis and progression of a neurodegenerative disorder, we performed longitudinal single-nucleus RNA sequencing of mouse and human spinocerebellar ataxia type 1 (SCA1) cerebellar tissue, establishing continuous dynamic trajectories of each cell population. Importantly, we defined the precise transcriptional changes that precede loss of Purkinje cells and, for the first time, identified robust early transcriptional dysregulation in unipolar brush cells and oligodendroglia. Finally, we applied a deep learning method to predict disease state accurately and identified specific features that enable accurate distinction of wild-type and SCA1 cells. Together, this work reveals new roles for diverse cerebellar cell types in SCA1 and provides a generalizable analysis framework for studying neurodegeneration.

2023-11-27 | GSE246183 | GEO

Cerebellar microglia-derived IL-17A compensates for autism-related deficits [cerebellar crus 1]

Project description:Interleukin-17 (IL-17) is a pleiotropic cytokine produced mainly by peripheral Th17 cells. Yet, brain functions of IL-17 derived from central nervous cells remain poorly understood. Here, we find an aberrant IL-17A signaling in the cerebellum of Fmr1-KO mice, a well-established genetic model for autism spectrum disorder (ASD). Cerebellar IL-17A, derived exclusively from microglia, is essential for the regulation of social behaviors by maintaining neuronal excitability and selectively suppressing inhibitory neurotransmission of Purkinje cells (PCs) in the cerebellar Crus I, a brain region critically involved in social cognition. Specific downregulation of IL-17 receptor-mediated signaling in cerebellar PCs recapitulates ASD-like social deficits and repetitive behaviors. Notably, both direct administration of IL-17A and induction of IL-17A release from cerebellar microglia by poly(I:C) effectively restore PC excitability and ameliorate ASD-like symptoms. The findings uncover an indispensable role of microglia-derived IL-17A for cerebellar social processing and suggest potential therapeutic strategies targeting IL-17A signaling for ASD.

2026-02-03 | GSE287731 | GEO

Cerebellar microglia-derived IL-17A compensates for autism-related deficits [purified cerebellar microglia]

2026-02-03 | GSE287705 | GEO

The Autism-Associated Protein CHD8 Is Required for Cerebellar Development and Motor Function

Project description:Mutations in the gene encoding the chromatin remodeler CHD8 are a highly penetrant risk factor for autism spectrum disorder (ASD). Although cerebellar abnormalities have been involved in ASD pathogenesis, the underlying mechanisms of CHD8 in the regulation of cerebellar function has remained unknown, however. Here we show that the cerebellar granule neurons–specific deletion of Chd8 manifests reduced proliferation and differentiation of granule neuron progenitors, leading to cerebellar hypoplasia and motor coordination defects in mice. CHD8 was found to regulate the expression of neuronal genes and to be required for maturation of cerebellar granule neurons. Furthermore, we found that CHD8 is preferentially bound to promoter regions and is necessary for modulation of local chromatin accessibilities at promoter regions of active gene. Our results thus uncover a critical role for CHD8 in cerebellar development and have important implications for understanding the cerebellar contributions to ASD pathogenesis.

2021-04-16 | GSE146885 | GEO

Epigenetic control of region specific transcriptional programs in mouse cerebellar and cortical astrocytes

Project description:Epigenetic control of region-specific transcriptional programs in mouse cerebellar and cortical astrocytes

| PRJEB31136 | ENA

Proteome analysis of mouse cerebellar synaptosomes

Project description:In this study we have compared the proteomes from cerebellar synaptosome of early symptomatic Cstb-/- and wild type mice to gain insight into disease onset and progression of cystatin B deficiency. Biallelic loss-of-function mutations in the CSTB gene cause progressive myoclonus epilepsy of Unverricht-Lundborg type (EPM1). In the mouse model of EPM1, CSTB deficiency manifests from one month of age as progressive neurodegeneration, myoclonus and ataxia, which is preceded by neuroinflammation, alterations in GABAergic signaling, and a widespread rearrangement of the mitochondrial proteome in synapses.

2023-07-20 | PXD040382 | Pride

Heterogeneity of Purkinje cells orchestrates cerebellar development.

Project description:Heterogeneity of Purkinje cells orchestrates cerebellar development.

| PRJNA1169626 | ENA

Heterogeneity of Purkinje cells orchestrates cerebellar development

Project description:Heterogeneity of Purkinje cells orchestrates cerebellar development

| PRJNA1079673 | ENA

Proteomic analysis of cerebellar synaptosomes in presymptomatic cystatin B –deficient mice

Project description:Loss-of-function mutations in cystatin B (CSTB) cause progressive myoclonus epilepsy of Unverricht-Lundborg type (EPM1). Cstb-deficiency in mice leads to early alterations in GABAergic signaling, and causes neuroinflammation followed by progressive neurodegeneration, manifesting as progressive myoclonus and ataxia. The proteome of cerebellar synaptosomes of presymptomatic Cstb-/- mice were characterized by LC-ESI-MS/MS to gain insight into disease formation and progression.

2020-10-20 | PXD019370 | Pride

OmicsDI is part of the ELIXIR infrastructure

OmicsDI is an Elixir interoperability service. Learn more ›

Tweets

OmicsDI Databases

PRIDE
PeptideAtlas
MassIVE
JPOST Repository
Physiome Model Repository

EGA
EVA
ENA
LINCS
PAXDB
Cell Collective

MetaboLights
Metabolomics Workbench
MetabolomeExpress
GNPS
BioModels
FAIRDOMHub

ArrayExpress
dbGaP
ExpressionAtlas
GEO
NODE

Information

Databases
Help
API
Contact us
Code on GitHub
Terms of use
Submit Data