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LORICRIN

Function

Major keratinocyte cell envelope protein.

Involvement in disease

Vohwinkel syndrome with ichthyosis

VSI

A variant form of Vohwinkel syndrome without hearing loss and associated with ichthyosiform dermatosis. Clinical features include palmoplantar keratoderma, pseudoainhum and ichthyosis. Compact hyperkeratosis with round retained nuclei and hypergranulosis is observed on skin biopsies.

None

The disease is caused by variants affecting the gene represented in this entry.

Post-translational modifications

Substrate of transglutaminases. Some glutamines and lysines are cross-linked to other loricrin molecules and to SPRRs proteins.

Contains inter- or intramolecular disulfide-bonds.

Cellular localization

Alternative names

LOR, LRN, LORICRIN, Loricrin

swissprot:P23490 omim:152445 entrezGene:4014