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ProteomicsLiver Steatosis and Mitochondrial Dysfunction in Spinal Muscular Atrophy is Survival Motor Neuron-Dependent and Hepatocyte-Intrinsic
Spinal Muscular Atrophy (SMA) is typically characterized as a motor neuron disease, but extra-neuronal phenotypes are present in almost every organ in severely affected patients and animal models. Extra-neuronal phenotypes were previously underappreciated as patients with severe SMA phenotypes usual...
ORGANISM(S): Homo Sapiens (human) 
2024-05-09 | PXD045401 | JPOST Repository
TranscriptomicsTranscriptomic profiling of spinal and bulbar muscular atrophy cultured embryonic motor neurons
Spinal and bulbar muscular atrophy (SBMA), also known as Kennedy’s Disease, is a slowly progressive adult-onset neuromuscular disease which results from a polyglutamine (polyQ) encoding CAG repeat expansion within the androgen receptor gene (AR). Despite the ubiquitous expression of the androgen rec...
ORGANISM(S): Mus musculus 
2019-05-02 | E-MTAB-6986 | biostudies-arrayexpress
TranscriptomicsMicroarray analysis of Gene expression in limb muscles of three mouse models of Kennedy disease/SBMA
Emerging evidence implicates transcriptional dyseregulation within skeletal muscle in the pathogenesis of Kennedy disease/spinal bulbar muscular atrophy (KD/SBMA). We therefore broadly characterized gene expression in skeletal muscle of three independently generated mouse models of this disorder. Th...
ORGANISM(S): Mus musculus 
2010-10-12 | E-GEOD-10190 | biostudies-arrayexpress
TranscriptomicsDNA damage signaling gene array on SMA mouse skeletal muscle
A commercially available gene array (SABiosciences array PAMM-029ZD-12) was used to assay gene expression in skeletal muscle of SMA mice and control littermates. The Taiwanese severe SMA mouse model was used (Hsieh-Li et al. 2000). The array included 84 genes associated with DNA damage detection, DN...
ORGANISM(S): Mus musculus 
2014-03-03 | E-GEOD-55481 | biostudies-arrayexpress
TranscriptomicsReal-time quantitative PCR analysis of rat diaphragm before and after acute cervical spinal cord injury
Male Sprague Dawley adult rats were subjected to a cervical hemisection at C2 (C2HS). Costal diaphragm (both ipsilesional and contralesional sides) were assessed under control conditions (sham surgery) and at 1 and 7 days post-C2HS. We used SA Biosciences Rat Skeletal Muscle Development and Disea...
ORGANISM(S): Rattus norvegicus 
2013-12-18 | E-GEOD-45021 | biostudies-arrayexpress
TranscriptomicsThe gene expression analysis of the spinal cord and skeletal muscle of SBMA (Spinal Bulbar Muscular Atrophy) model mice with or without pioglitazone (PG) treatment
To identify the gene expression changes by administering PG to SBMA model mice, we prepared total RNA samples from the spinal cords and skeletal muscles of transgenic mice carrying a full-length human AR with 97 CAGs (AR-97Q) that were treated with or without PG. We used AR-97Q (Line #7-8) male mice...
ORGANISM(S): Mus musculus 
2013-10-29 | E-GEOD-51807 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of muscular and neuronal mouse mutant: spinal muscular atrophy(SMA)
Study of gene expression profiles of muscular and neuronal mouse mutant of spinal muscular atrophy(SMA). Pre and post symptomatic stage disease have been analyzed.
ORGANISM(S): Mus musculus 
2005-05-20 | E-MEXP-131 | biostudies-arrayexpress
TranscriptomicsEffect of fasting on human skeletal muscle mRNA levels
The goal of these studies was to determine the effects of fasting on skeletal muscle mRNA levels in healthy human subjects. Seven healthy adult human subjects fasted for 40 hours and then a muscle biopsy (fasting sample) was obtained from the vastus lateralis muscle. Immediately after the first mus...
ORGANISM(S): Homo sapiens 
2011-06-08 | E-GEOD-28016 | biostudies-arrayexpress
TranscriptomicsRNA-seq transcriptome profiling of motor neurons (MNs) and surrounding white matter glia (WM) microdissected from pre-symptomatic mouse model of spinal muscular atrophy (SMA)
We report tramscriptome perturbations in MNs and WM of SMA mouse, prior to first morphological and biochemical synaptic defects Examination of ventral horn MNs and WM isolated from lumbar segment spinal cords from wild type (WT) and SMA mice
ORGANISM(S): Mus musculus 
2013-11-05 | E-GEOD-51735 | biostudies-arrayexpress
TranscriptomicsThe gene expression analysis of the spinal cord of SBMA (Spinal Bulbar Muscular Atrophy) model mice
To identify the gene expression changes that are specific to SBMA, we prepared total mRNA samples from the spinal cords of transgenic mice carrying a full-length human AR with 97 CAGs (AR-97Q), transgenic mice bearing a wild-type allele of AR with 24 CAGs (AR-24Q), and the wild-type littermates of t...
ORGANISM(S): Mus musculus 
2012-08-03 | E-GEOD-39865 | biostudies-arrayexpress
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