GEO DataSets

(Submitter supplied) Analysis of cerebella from Capicua (Cic) mutant mice and wild-type controls at 28 days of age (P28). Spinocerebellar ataxia type 1 (SCA1) is a fatal neurodegenerative disease caused by expansion of a translated CAG repeat in Ataxin-1 (ATXN1). The transcriptional repressor Cic binds directly to Atxn1 and plays a key role in SCA1 pathogenesis. Two isoforms of Cic, long (Cic-L) and short (Cic-S), are transcribed from alternative promoters. more...

Organism:

Mus musculus

Type:

Expression profiling by array

Platform:

GPL10740

8 Samples

Download data: CEL

(Submitter supplied) This SuperSeries is composed of the SubSeries listed below.

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL13112

22 Samples

Download data: TXT

(Submitter supplied) We isolated RNA from cerebella dissected from Pcp2-ATXN1[82Q], Pcp2-ATXN1[82Q]V591A;S602D, and wild-type littermates at one year of age

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL13112

15 Samples

Download data: TXT

(Submitter supplied) We isolated RNA from cerebella dissected from En1-Cre; Cicflox/flox and Cicflox/+ littermates at one year of age

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL13112

7 Samples

Download data: TXT

(Submitter supplied) RNA was isolated from mouse cerebellum at 6 weeks of age in 5 different gentoypes; wild-type (WT), Atxn1_154Q/2Q (SCA1), Atxn1_154Q[S776A]/2Q (SCA1 S776A), Atxn1_154Q[S776A]/2Q[S776A] (S776A Double) and Atxn1_2Q[S776A]/2Q[S776A] (homo). After RNA isolation, RNA-seq was performed and gene expression profiles were compared between WT, SCA1, and the S776A mutants. The goal was to determine if mutating the phosphorylation site S776 in the context of spinocerebellar ataxia type 1 (SCA1) is protective.

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL21103

24 Samples

Download data: TAB

(Submitter supplied) Comparative analysis of cerebellar gene expression changes occurring in Sca1154Q/2Q and Sca7266Q/5Q knock-in mice Polyglutamine diseases are inherited neurodegenerative disorders caused by expansion of CAG repeats encoding a glutamine tract in the disease-causing proteins. There are nine disorders each having distinct features but also clinical and pathological similarities. In particular, spinocerebellar ataxia type 1 and type 7 (SCA1 and SCA7) patients manifest cerebellar ataxia with degeneration of Purkinje cells. more...

Organism:

Mus musculus

Type:

Expression profiling by array

Datasets:

GDS3544 GDS3545

Platforms:

GPL339 GPL1261

22 Samples

Download data: CEL

Analysis of cerebella from spinocerebellar ataxia type 7 (SCA7) knockin animals at an early symptomatic disease stage. SCA1 is a neurodegenerative disorder caused by expansion of a glutamine tract in ataxin-7. Results provide insight into the molecular basis of cerebellar pathogenesis in SCA7.

Organism:

Mus musculus

Type:

Expression profiling by array, transformed count, 2 genotype/variation sets

Platform:

GPL1261

Series:

GSE9914

10 Samples

Download data: CEL

Analysis of cerebella from spinocerebellar ataxia type 1 (SCA1) knockin animals at an early symptomatic disease stage. SCA1 is a neurodegenerative disorder caused by expansion of a glutamine tract in ataxin-1. Results provide insight into the molecular basis of cerebellar pathogenesis in SCA1.

Organism:

Mus musculus

Type:

Expression profiling by array, transformed count, 3 age, 2 genotype/variation sets

Platform:

GPL339

Series:

GSE9914

12 Samples

Download data: CEL

(Submitter supplied) SCA1, a fatal neurodegenerative disorder, is caused by a CAG expansion encoding a polyglutamine stretch in the protein ATXN1. We used RNA-seq to profile cerebellar RNA expression in ATXN1 mice, including lines with ataxia and progressive pathology and lines having ataxia in absence of Purkinje cell progressive pathology. Weighted Gene Coexpression Network Analysis of the cerebellar RNA-seq data revealed two gene networks that significantly correlated with disease, the Magenta (342 genes) and Light Yellow (35 genes) Modules. more...

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platforms:

GPL11002 GPL13112

30 Samples

Download data: TXT

(Submitter supplied) This SuperSeries is composed of the SubSeries listed below.

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing; Genome binding/occupancy profiling by high throughput sequencing

Platforms:

GPL24247 GPL17021

104 Samples

Download data: BW, TAB

(Submitter supplied) Genome wide binding profiles of CIC and H3K27ac in CIC KO (Engrailed1-Cre;Cicfl/fl), wildtype, Atxn1_154Q/2Q (SCA1), and Atxn1_154Q[V5591A;S602D]/2Q (154Q AXH) in the cerebellum

Organism:

Mus musculus

Type:

Genome binding/occupancy profiling by high throughput sequencing

Platform:

GPL24247

45 Samples

Download data: BW, NARROWPEAK

(Submitter supplied) mRNA profiles of 10-week mice in wild-type (WT), Atxn1_154Q/2Q (SCA1), Atxn1_154Q[V5591A;S602D]/2Q (154Q AXH) genotypes across 5 different brain regions (cerebellum, brainstem, hippocampus, striatum and cortex)

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL17021

59 Samples

Download data: TAB

(Submitter supplied) Although expansion of a polyglutamine tract in ATAXIN1 (ATXN1) causes Spinocerebellar ataxia type 1, the functions of wild-type ATXN1 and ATAXIN1-Like (ATXN1L) remain poorly understood. To gain insight into the function of these proteins, we generated and characterized Atxn1L-/- and Atxn1-/- ; Atxn1L-/- double mutant animals. We found that Atxn1L -/- mice have several developmental problems including hydrocephalus, omphalocoele and lung alveolarization defects. more...

Organism:

Mus musculus

Type:

Expression profiling by array

Datasets:

GDS4148 GDS4149

Platform:

GPL11078

14 Samples

Download data: CEL

Analysis of lung of ATAXIN1-Like-/- (Atxn1L-/-) C57BL/6J mice at P6. Alveolarization defects causing air space enlargement were apparent in ATXN1L knockouts at late alveolar stage (P17-23). Results provide insight into the molecular mechanisms mediating the alveolarization defect.

Organism:

Mus musculus

Type:

Expression profiling by array, transformed count, 2 genotype/variation sets

Platform:

GPL11078

Series:

GSE29551

8 Samples

Download data: CEL

Analysis of lung of Atxn1-/- C57BL/6J mice at P6. Alveolarization defects causing air space enlargement were apparent in paralog of ATXN1 (ATAXIN1-Like) knockouts (Atxn1L-/-) at late alveolar stage (P17-23). Results provide insight into the molecular mechanisms mediating the alveolarization defect.

Organism:

Mus musculus

Type:

Expression profiling by array, transformed count, 2 genotype/variation sets

Platform:

GPL11078

Series:

GSE29551

6 Samples

Download data: CEL

(Submitter supplied) We performed an shRNA screen to identify novel ATXN1 protein level regulators in hope of finding some that may improve brainstem function in SCA1. We found that two closely related BTB-ZF transcription factors, ZBTB7A and ZBTB7B, positively regulate ATXN1 levels in vitro and in vivo, with ZBTB7B displaying a more pronounced effect. ZBTB7B regulates ATXN1 by regulating the transcription of RSK3 (RPS6KA2). more...

Organism:

Homo sapiens

Type:

Expression profiling by high throughput sequencing

Platform:

GPL11154

6 Samples

Download data: TXT

(Submitter supplied) We isolated RNAs from Otp-lineage cells in the mouse brain using translating ribosome affinity purification (TRAP) approach. We compared two groups of mice: Otp-cre; ROSAfsTRAP (control) and Otp-cre; Cicf/f; ROSAfsTRAP (conditional knockout). Following RNA isolation, RNAseq was performed and gene expression profiles were compared between controls and conditional knockouts.

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL17021

8 Samples

Download data: TXT

(Submitter supplied) RNA-targeting approaches have improved disease symptoms in preclinical rodent models of several neurological diseases. Spinocerebellar ataxia type 1 (SCA1) is a dominantly inherited ataxia caused by expansion of a polyglutamine tract in the encoded protein Ataxin-1 (ATXN1). Despite advances in understanding this CAG repeat/polyglutamine expansion disease, there are still no therapies to alter its progressive fatal course. more...

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL17021

65 Samples

Download data: TXT