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Chemical chaperones protect epidermolysis bullosa simplex keratinocytes from heat stress-induced keratin aggregation : involvement of heat shock proteins and MAP kinasesChamcheu, Jean Christopher ...Epidermolysis bullosa simplex (EBS) is a blistering skin disease caused by mutations in keratin genes (KRT5 or KRT14), with no existing therapies. Aggregates of misfolded mutant keratins are seen in ... cultured keratinocytes from severe EBS patients. In other protein-folding disorders, involvement of molecular chaperones and the ubiquitin-proteasome system may modify disease severity. In this study, the effects of heat stress on keratin aggregation in immortalized cells from two patients with EBS (KRT5) and a healthy control were examined with and without addition of various test compounds. Heat-induced (43 °C, 30 minutes) aggregates were observed in all cell lines, the amount of which correlated with the donor phenotype. In EBS cells pre-exposed to proteasome inhibitor, MG132, and p38-mitogen-activated protein kinase (MAPK) inhibitor, SB203580, the proportion of aggregate-positive cells increased, suggesting a role of proteasomes and phosphorylation in removing mutated keratin. In contrast, aggregates were reduced by pretreatment with twochemical chaperones, trimethylamine N-oxide (TMAO) and 4-phenylbutyrate (4-PBA). TMAO also modulated stress-induced p38/c-jun N-terminal kinase (JNK) activation and expression of heat shock protein (HSPA1A), the latter of which colocalized with phosphorylated keratin 5 in EBS cells. Taken together, our findings suggest therapeutic targets for EBS and other keratinopathies.Vir: The Journal of investigative dermatology. - ISSN 0022-202X (Vol. 131, issue 8, 2011, str. 1684-1691)Vrsta gradiva - članek, sestavni delLeto - 2011Jezik - angleškiCOBISS.SI-ID - 28273369
Avtor
Chamcheu, Jean Christopher |
Navsaria, Harshad |
Pihl-Lundin, Inger |
Liović, Mirjana |
Vahlquist, Anders |
Törmä, Hans
Teme
Epidermolysis Bullosa Simplex |
Keratinocytes |
Heat-Shock Proteins |
Molecular Chaperones |
Keratin |
Genetics |
Mutation |
Protein Folding |
Heat-Shock Response |
Cell Line |
Protein Kinases |
Antagonists And Inhibitors |
Phosphorylation |
Keratinociti |
Epidermoliza bulozna, simpleksna |
Molekularni haperoni |
Keratin |
Vročinski-šok beljakovine |
Celična linija |
Vročinski šok, odgovor |
Fosforilacija |
Beljakovine, gubanje |
Mutacija |
Beljakovinske kinaze
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Chamcheu, Jean Christopher | ![]() |
Navsaria, Harshad | ![]() |
Pihl-Lundin, Inger | ![]() |
Liović, Mirjana | 14305 |
Vahlquist, Anders | ![]() |
Törmä, Hans | ![]() |
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