The parathyroid hormone family member TIP39 interacts with sarco/endoplasmic reticulum Ca²⁺ - ATPase activity by influencing calcium homoeostasis.

ABSTRACT: Darier disease (DD) is a genetic skin disease that is associated with mutations in the ATP2A2 gene encoding the type 2 sarco/endoplasmic reticulum (ER) Ca²⁺ - ATPase (SERCA2). Mutations of this gene result in alterations of calcium homoeostasis, abnormal epidermal adhesion and dyskeratosis. Silencing of ATP2A2 in monolayer cell culture of keratinocytes reduces desmoplakin expression at the borders of cells and impacts cell adhesion. Here, we report establishment of a three-dimensional (3D) epidermal model of DD and use this model to evaluate peptide therapy with tuberoinfundibular peptide of 39 residues (TIP39) to normalize calcium transport. Gene silencing of ATP2A2 in keratinocytes grown in a 3D model resulted in dyskeratosis, partial parakeratosis and suprabasal clefts that r

SUBMITTER: Sato E 

PROVIDER: S-EPMC5513796 | biostudies-literature | 2017 Sep

REPOSITORIES: biostudies-literature