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Characterization of...
Characterization of immortalized human epidermolysis bullosa simplex (KRT5) cell lines : trimethylamine N-oxide protects the keratin cytoskeleton against disruptive stress condition
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- Chamcheu, Jean Christopher (author)
- Uppsala universitet,Dermatologi och venereologi,Dermatology and Venereology
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- Lorié, Elizabeth Pavez (author)
- Uppsala universitet,Dermatologi och venereologi,Dermatologi och venereologi, Dermatology and Venereology
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- Akgul, Baki (author)
- Centre for Cutaneous Research, ICMS, Bart's and London Queen Mary's School of Medicine and Dentistry, London, United Kingdom
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- Bannbers, Elin (author)
- Uppsala universitet,Dermatologi och venereologi
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- Virtanen, Marie (author)
- Uppsala universitet,Dermatologi och venereologi,Dermatology and Venereology
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- Gammon, Luke (author)
- Centre for Cutaneous Research, ICMS, Bart's and London Queen Mary's School of Medicine and Dentistry, London, United Kingdom
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- Moustakas, Aristidis (author)
- Uppsala universitet,Ludwiginstitutet för cancerforskning
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- Navsaria, Harshad (author)
- Centre for Cutaneous Research, ICMS, Bart's and London Queen Mary's School of Medicine and Dentistry, London, United Kingdom
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- Vahlquist, Anders (author)
- Uppsala universitet,Dermatologi och venereologi,Dermatology and Venereology
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- Törmä, Hans (author)
- Uppsala universitet,Dermatologi och venereologi,Dermatology and Venereology
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(creator_code:org_t)
- Elsevier, 2009
- 2009
- English.
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In: Journal of dermatological science (Amsterdam). - : Elsevier. - 0923-1811 .- 1873-569X. ; 53:3, s. 198-206
- Related links:
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
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- BACKGROUND: Epidermolysis bullosa simplex (EBS) is an autosomal inherited mechano-bullous disease, characterized by intraepidermal blistering and skin fragility caused by mutations in the keratin (KRT) 5 or 14 genes. Despite a vast knowledge about the intermediate filament pathology in this disease, the progress in therapy has been slow. Animal models and well-characterized continuous cell culture models of EBS are needed prior to clinical testing. OBJECTIVES: Our aim was to generate immortalized cell lines as an in vitro model for the study of EBS and test a chemical chaperone, trimethylamine N-oxide (TMAO), as a putative novel therapy. METHODS: We generated four immortalized cell lines, two each from an EBS patient with a KRT5-mutation (V186L) and a healthy control, using human papillomavirus 16 (HPV16) E6E7 as transducer. Cell lines were established in serum-free and serum-containing medium and assessed for growth characteristics, keratin expression profiles, ability to differentiate in organotypic cultures, and response to heat stress with and without the presence of TMAO. RESULTS: All cell lines have been expanded >160 population doublings and their cellular characteristics are similar. However, the formation of cytoplasmic keratin filament aggregates in response to heat-shock treatment differed between EBS and normal cell lines. Notably, serum-free established EBS-cell line was most vulnerable to heat shock but both cell lines exhibited significant reduction in the number of keratin aggregates containing cells by TMAO. CONCLUSION: The immortalized cell lines represent a suitable model for studying novel therapies for EBS. TMAO is a promising new agent for future development as a novel EBS therapy.
Keyword
- Chemical chaperone
- Cytoprotection
- EBS-cell lines
- Genetic skin disorder
- Human papilloma virus 16 E6/E7
- Heat stress
- Keratin
- Organotypic epidermis
- TMAO
- MEDICINE
- MEDICIN
- Dermatology and Venerology
- Dermatologi och venereologi
Publication and Content Type
- ref (subject category)
- art (subject category)
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Chamcheu, Jean C ...
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Lorié, Elizabeth ...
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Akgul, Baki
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Bannbers, Elin
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Virtanen, Marie
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Gammon, Luke
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Moustakas, Arist ...
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Navsaria, Harsha ...
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Vahlquist, Ander ...
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Törmä, Hans
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Journal of derma ...
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Uppsala University