E-MTAB-1640 - Transcription profiling by array of two HPV16 E6/E7 immortalized skin keratinocyte cell lines, NEB1 and KEB7, derived from a healthy individual and a patient suffering from Dowling-Meara type epidermolysis bullosa simplex (EBS-DM), respectively

Status
Released on 23 July 2013, last updated on 3 June 2014
Organism
Homo sapiens
Samples (2)
Array (1)
Protocols (6)
Description
Dowling-Meara type epidermolysis bullosa simplex (EBS-DM) is a severe blistering disease, caused by dominant mutations in either the keratin-5 (K5) or keratin-14 (K14) gene. K5 and K14 are the major components of the intermediate filament (IF) network in basal keratinocytes. Due to the dominant nature of EBS-DM, misfolded K5 or K14 proteins are incorporated into intermediate filaments, rendering them sensitive to mechanical stress. Upon trauma, these filaments disrupt and the keratinocytes lyse, leading to intra-epidermal blistering. The dominant nature of K5 and K14 mutations in EBS-DM represents a challenge to gene therapeutic approaches. Therefore, we investigated the gene expression profile of a K14 mutant keratinocyte cell line (KEB7) and compared it to the gene expression profile of a wild-type keratinocyte cell line (NEB1). The aim of this study was to identify differentially regulated genes as potential therapeutic targets for the development of new therapies.
Experiment types
transcription profiling by array, cell type comparison, co-expression, in vitro
Contact
Citation
MMP-9 and CXCL8/IL-8 Are Potential Therapeutic Targets in Epidermolysis Bullosa Simplex . Lettner T, Lang R, Klausegger A, Hainzl S, Bauer JW and Wally V. PLoS ONE 8(7):1-14 (2013)
MIAME
PlatformsProtocolsFactorsProcessedRaw
Files
Investigation descriptionE-MTAB-1640.idf.txt
Sample and data relationshipE-MTAB-1640.sdrf.txt
Raw data (1)E-MTAB-1640.raw.1.zip
Processed data (2)E-MTAB-1640.processed.1.zip, E-MTAB-1640.processed.2.zip
Array designA-AFFY-168.adf.txt
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