Dataset Information


Transcription profiling by array of human olfactory ecto-mesenchymal stem cells in familial dysautonomia

ABSTRACT: Exploration of transcriptome expression in 5 control and 4 familial dysautonomia (FD) human olfactory ecto-mesenchymal stem cells (hOE-MSCs) at very early (P1 and P2) and later (P5 and P9) cell passages.

ORGANISM(S): Homo sapiens  

DISEASE(S): Reference,Familial Dysautonomia

SUBMITTER: Ibrahim El Chérif  

PROVIDER: E-MTAB-281 | ArrayExpress | 2011-01-01


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Olfactory stem cells, a new cellular model for studying molecular mechanisms underlying familial dysautonomia.

Boone Nathalie N   Loriod Béatrice B   Bergon Aurélie A   Sbai Oualid O   Formisano-Tréziny Christine C   Gabert Jean J   Khrestchatisky Michel M   Nguyen Catherine C   Féron François F   Axelrod Felicia B FB   Ibrahim El Chérif EC  

PloS one 20101220 12

BACKGROUND: Familial dysautonomia (FD) is a hereditary neuropathy caused by mutations in the IKBKAP gene, the most common of which results in variable tissue-specific mRNA splicing with skipping of exon 20. Defective splicing is especially severe in nervous tissue, leading to incomplete development and progressive degeneration of sensory and autonomic neurons. The specificity of neuron loss in FD is poorly understood due to the lack of an appropriate model system. To better understand and modeli  ...[more]

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