Unknown,Transcriptomics,Genomics,Proteomics

Dataset Information

RNA-seq transcriptome profiling of motor neurons (MNs) and surrounding white matter glia (WM) microdissected from pre-symptomatic mouse model of spinal muscular atrophy (SMA)

ABSTRACT: 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

SUBMITTER: Zhenxi Zhang

PROVIDER: E-GEOD-51735 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Publications

Dysregulation of synaptogenesis genes antecedes motor neuron pathology in spinal muscular atrophy.

Zhang Zhenxi Z Pinto Anna Maria AM Pinto Anna Maria AM Wan Lili L Wang Wei W Berg Michael G MG Oliva Isabela I Singh Larry N LN Dengler Christopher C Wei Zhi Z Dreyfuss Gideon G

Proceedings of the National Academy of Sciences of the United States of America 20131104 48

The motor neuron (MN) degenerative disease, spinal muscular atrophy (SMA) is caused by deficiency of SMN (survival motor neuron), a ubiquitous and indispensable protein essential for biogenesis of snRNPs, key components of pre-mRNA processing. However, SMA's hallmark MN pathology, including neuromuscular junction (NMJ) disruption and sensory-motor circuitry impairment, remains unexplained. Toward this end, we used deep RNA sequencing (RNA-seq) to determine if there are any transcriptome changes ...[more]

PMID: 24191055

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Project description:Coeliac disease is a small intestinal disorder caused by an abarrent immune response towards dietary gluten due to activation of pro-inflammatory gluten specific CD4+ T cells. Histological evaluation and classification of gluten induced musosal changes is part of the diagnostic work up. of adults. The kinetics of mucosal recovery following commencesment of a gluten free diet (disease remission) and the degree of mucosal changes induced by gluten reintroduction (gluten challenge) varies between patients. Also, patients classified with similar clinical and histological disease remission, can develop different degree of mucosal damage following the same gluten challenge regime. This variation poses a challenge for the interpretation of gluten induced mucosal changes both in a diagnostic and clinical trial settings. In this study, we have analysed material from small intestinal biopsies collected from 19 treated coeliac disease patients before and after completion of a 14-day oral gluten challenge. These patients are part of a previoslpreviouslyu described study where all patients were in clinical and mucosal remission at baseline but only some patients developed histological changes in the mucosa in response to gluten. We have performed shotgun LC-MSMS analysis and label-free quantification of total gut tissue and from laser capture microdissected epithelial cell layer samples. We found that differences in tissue proteome expression could separate patients as responders and non-responders to the gluten challenge. Patients whoich responded strongly to gluten , had signs of gut inflammation already at baseline, supported by presence of low-level blood inflammatory parameters and a slight increase in numbers of gluten-specific CD4+ T cells at baseline. Our proteomics analysis demonstrated baseline differences in gut tissue state between patients that were not evident from routine clinical and histological evaluation. These baseline differences likely explains why some patients respond more strongly to gluten challenge than others.

Dataset Information

RNA-seq transcriptome profiling of motor neurons (MNs) and surrounding white matter glia (WM) microdissected from pre-symptomatic mouse model of spinal muscular atrophy (SMA)

Publications

Dysregulation of synaptogenesis genes antecedes motor neuron pathology in spinal muscular atrophy.

Similar Datasets

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