RGD Reference Report - Interleukin-1 alpha blockade prevents hyperkeratosis in an in vitro model of lamellar ichthyosis. - Rat Genome Database

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Interleukin-1 alpha blockade prevents hyperkeratosis in an in vitro model of lamellar ichthyosis.

Authors: O'Shaughnessy, RF  Choudhary, I  Harper, JI 
Citation: O'Shaughnessy RF, etal., Hum Mol Genet. 2010 Jul 1;19(13):2594-605. Epub 2010 Apr 12.
RGD ID: 4142790
Pubmed: PMID:20385541   (View Abstract at PubMed)
DOI: DOI:10.1093/hmg/ddq145   (Journal Full-text)

The autosomal recessive congenital ichthyoses are a family of related diseases, causing a severe defect in the barrier function of the epidermis. Neonates are usually born as collodion babies, but later form scales characteristic of the disease, due to a combination of thickening of the cornified layer and an increase in the production of non-polar lipids. Current treatments of choice are exfoliative creams and moisturizing agents and the use of oral retinoids. The skin condition and treatment impact significantly on quality of life and, with oral retinoids, there are potential complications associated with long-term use. A greater understanding of the mechanisms that result in scaling should lead to better directed therapies, not only for the inherited ichthyoses, but also other hyperkeratotic disorders. Using siRNA knockdown of the principle gene mutated in lamellar ichthyosis (LI), transglutaminase-1, in rat keratinocytes, we created an in vitro organotypic culture model that closely mimics the disease. Interleukin-1 alpha (IL1A) expression was increased and there was a lack of loricrin cross-linking. All LI patients tested had an increased IL1A and treatment of wild-type organotypic cultures with IL1A was sufficient to induce hyperkeratosis. Treatment of disease mimic organotypic cultures with IL-1 receptor antagonist led to a dose-dependent decrease in hyperkeratosis without a reduction in non-polar lipids in the cornified layer, which has the potential to reduce scaling without the requirement to constantly apply emollients.



Gene Ontology Annotations    Click to see Annotation Detail View

Biological Process

  
Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
Il1aRatkeratinization  IDA  RGD 

Objects Annotated

Genes (Rattus norvegicus)
Il1a  (interleukin 1 alpha)

Objects referenced in this article
Gene IL1A interleukin 1 alpha Homo sapiens
Gene Il1a interleukin 1 alpha Mus musculus

Additional Information