Profilaggrin is a major protein component of the keratohyalin granules of mammalian epidermis. It is initially expressed as a large polyprotein precursor which is subsequently proteolytically processed into individual functional filaggrin molecules.

The filaggrins show wide species variations and their aberrant expression has been implicated in a number of keratinizing disorders, like ichthyosis vulgaris.

Pathology

 ichthyosis vulgaris

See also

 IVL
 LOR

References

 Smith FJ, Irvine AD, Terron-Kwiatkowski A, Sandilands A, Campbell LE, Zhao Y, Liao H, Evans AT, Goudie DR, Lewis-Jones S, Arseculeratne G, Munro CS, Sergeant A, O'regan G, Bale SJ, Compton JG, Digiovanna JJ, Presland RB, Fleckman P, McLean WH. Loss-of-function mutations in the gene encoding filaggrin cause ichthyosis vulgaris.Nat Genet. 2006 Jan 29; PMID: #16444271#