Epiplakin

EPPK1
Identifiers
AliasesEPPK1, EPIPL, EPIPL1, epiplakin 1
External IDsOMIM: 607553; MGI: 2386306; HomoloGene: 20006; GeneCards: EPPK1; OMA:EPPK1 - orthologs
Orthologs
SpeciesHumanMouse
Entrez

83481

223650

Ensembl

ENSG00000261150

ENSMUSG00000115388

UniProt

P58107

Q8R0W0

RefSeq (mRNA)

NM_031308

NM_144848

RefSeq (protein)

NP_112598

NP_659097

Location (UCSC)Chr 8: 143.86 – 143.88 Mbn/a
PubMed search[2][3]
Wikidata
View/Edit HumanView/Edit Mouse

Epiplakin is a large cytoplasmic protein that is encoded by the EPPK1 gene in humans. Epiplakin was first identified as an autoantigen.[4]

Discovery

The initial discovery of Epiplakin came from a patient who had a rare autoimmune skin disease that caused blistering at the junction of the epidermis and dermis. After closer examination, scientists saw that the patient's blood had contained autoantibodies that reacted with an unknown protein in the epidermis. The unknown protein was almost entirely made of repeated plakin domains.[4]

Subcellular distribution

The peptide recognition domain of epiplakin is able to bind to keratins in vitro; however, in cells the epiplakin associates with keratin intermediate filaments networks only under conditions in which cellular stress is absent. Otherwise, epiplakin remains universally cytoplasmic and not bound to the intermediate filaments.

Structure

Human epiplakin contains 13 peptide recognition domains (PRDs) and have a total size of about 725 kDa. Unlike plakins, they lack an N-terminal actin-binding domain.[5][4]

Function

Epiplakin's role in maintaining keratin intermediate filament organization suggests that dysregulation of epiplakin expression or function may contribute to epithelial fragility or altered wound-healing responses, as indicated by accelerated wound closure observed in epiplakin-depleted corneal epithelial cells.

Clinical significance

Cancer

Scientists have examined EPPK1 expression in multiple types/forms of cancers (bladder, lung, colon, etc.). Various studies show that altered Epiplakin levels in tumor tissues show correlation with tumor progression pathways.[6]

References

  1. ^ a b c GRCh38: Ensembl release 89: ENSG00000261150Ensembl, May 2017
  2. ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  3. ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ^ a b c Fujiwara S, Takeo N, Otani Y, Parry DA, Kunimatsu M, Lu R, et al. (April 2001). "Epiplakin, a novel member of the Plakin family originally identified as a 450-kDa human epidermal autoantigen. Structure and tissue localization". The Journal of Biological Chemistry. 276 (16): 13340–13347. doi:10.1074/jbc.M011386200. PMID 11278896.
  5. ^ Spazierer D, Fuchs P, Pröll V, Janda L, Oehler S, Fischer I, et al. (August 2003). "Epiplakin gene analysis in mouse reveals a single exon encoding a 725-kDa protein with expression restricted to epithelial tissues". The Journal of Biological Chemistry. 278 (34): 31657–31666. doi:10.1074/jbc.M303055200. PMID 12791695.
  6. ^ Shimura S, Matsumoto K, Shimizu Y, Mochizuki K, Shiono Y, Hirano S, et al. (October 2021). "Serum Epiplakin Might Be a Potential Serodiagnostic Biomarker for Bladder Cancer". Cancers. 13 (20): 5150. doi:10.3390/cancers13205150. PMC 8534213. PMID 34680299.

Further reading