RGD Reference Report - Peripheral myelin protein 22 is a constituent of intercellular junctions in epithelia. - Rat Genome Database

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Peripheral myelin protein 22 is a constituent of intercellular junctions in epithelia.

Authors: Notterpek, L  Roux, KJ  Amici, SA  Yazdanpour, A  Rahner, C  Fletcher, BS 
Citation: Notterpek L, etal., Proc Natl Acad Sci U S A 2001 Dec 4;98(25):14404-9.
RGD ID: 70581
Pubmed: PMID:11717414   (View Abstract at PubMed)
PMCID: PMC64694   (View Article at PubMed Central)
DOI: DOI:10.1073/pnas.251548398   (Journal Full-text)

Alterations in peripheral myelin protein 22 (PMP22) gene expression are associated with a host of heritable demyelinating peripheral neuropathies, yet the function of the protein remains unknown. PMP22 expression is highest in myelinating Schwann cells of peripheral nerves; however, significant levels of PMP22 mRNAs can be detected in a variety of non-neural tissue, including epithelia. To date, PMP22 protein expression and localization in non-neural tissues have not been studied in detail. In adult rat liver and intestine, and cultured epithelial cells, we detected PMP22-like immunoreactivity associated with markers of the tight junctional complex, including zonula occludens 1 (ZO-1) and occludin. Upon disruption of intercellular contacts, PMP22 was internalized into vesicles that were immunoreactive for both anti-occludin and anti-PMP22 antibodies. Nonionic detergent extraction of cultured epithelial cells did not solubilize PMP22, as the majority of the protein remained in the detergent insoluble fraction, as did ZO-1 and occludin. We also observed the targeting of exogenous myc-tagged PMP22 to apical cell junctions in polarized epithelia and to anti-ZO-1 antibody immunoreactive cell contacts of L fibroblasts. These studies support a role for PMP22 at intercellular junctions of epithelia and may indicate a similar function in myelinating Schwann cells. Furthermore, our findings could provide an explanation for certain phenotypes of PMP22 neuropathy mice that cannot be accounted for by dysmyelination.

Gene Ontology Annotations    Click to see Annotation Detail View

Cellular Component
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
bicellular tight junction  IDA 70581 RGD 

Objects Annotated

Genes (Rattus norvegicus)
Pmp22  (peripheral myelin protein 22)


Additional Information