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Series GSE30960 Query DataSets for GSE30960
Status Public on Feb 23, 2012
Title Expression analysis of pre-phenotype dt27J mouse dorsal root ganglia
Organism Mus musculus
Experiment type Expression profiling by array
Summary Loss of function of dystonin cytoskeletal linker proteins causes neurodegeneration in the sensory ataxia, dystonia musculorum (dt). While much investigation has focused on understanding dt pathology, divergent functions of dystonin isoforms are still unknown. Here, we highlight a novel function of the dystonin-a2 isoform in mediating microtubule (MT) stability, golgi organization and flux through the secretory pathway. Using dystonin-null mice combined with isoform-specific loss of function analysis, we find dystonin-a2 is bound to MAP1B in the area surrounding the centrosome, where it maintains MT acetylation. In dt, the absence of the MAP1B-dystonin-A2 interaction results in a loss of MAP1B perinuclear localization, leading to MT deacetylation and instability. Deacetylated MTs lead to golgi fragmentation and prevent anterograde trafficking of motor proteins. Maintenance of MT acetylation through TSA administration or MAP1B overexperssion in vitro, mitigates the observed defect. These aberrations are apparent in pre-phenotype dorsal root ganglia (DRG) and primary sensory neurons, suggesting they are causal in the dt disorder.
Overall design P4 dorsal root ganglia (DRG) tissue from 3 WT and 3 dt27J animals from 2 separate litters was subjected to RNA extraction and analyzed.
Contributor(s) Ryan SD, Chu A, Blais A, Kothary R
Citation(s) 22412020
Submission date Jul 26, 2011
Last update date Jan 12, 2017
Contact name Scott D Ryan
Organization name Ottawa Hospital Research Institute
Department Regenerative Medicine
Lab Kothary Lab
Street address 501 Smyth Rd
City Ottawa
State/province ON
ZIP/Postal code K1H 8L6
Country Canada
Platforms (1)
GPL7202 Agilent-014868 Whole Mouse Genome Microarray 4x44K G4122F (Probe Name version)
Samples (6)
GSM767652 WT, rep1
GSM767653 WT, rep2
GSM767654 WT, rep3
BioProject PRJNA146157

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Supplementary file Size Download File type/resource
GSE30960_RAW.tar 9.2 Mb (http)(custom) TAR (of TXT)
Processed data included within Sample table

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