[HTML][HTML] Desmoglein-1/Erbin interaction suppresses ERK activation to support epidermal differentiation

RM Harmon, CL Simpson, JL Johnson… - The Journal of …, 2013 - Am Soc Clin Investig
RM Harmon, CL Simpson, JL Johnson, JL Koetsier, AD Dubash, NA Najor, O Sarig…
The Journal of clinical investigation, 2013Am Soc Clin Investig
Genetic disorders of the Ras/MAPK pathway, termed RASopathies, produce numerous
abnormalities, including cutaneous keratodermas. The desmosomal cadherin, desmoglein-1
(DSG1), promotes keratinocyte differentiation by attenuating MAPK/ERK signaling and is
linked to striate palmoplantar keratoderma (SPPK). This raises the possibility that cutaneous
defects associated with SPPK and RASopathies share certain molecular faults. To identify
intermediates responsible for executing the inhibition of ERK by DSG1, we conducted a …
Genetic disorders of the Ras/MAPK pathway, termed RASopathies, produce numerous abnormalities, including cutaneous keratodermas. The desmosomal cadherin, desmoglein-1 (DSG1), promotes keratinocyte differentiation by attenuating MAPK/ERK signaling and is linked to striate palmoplantar keratoderma (SPPK). This raises the possibility that cutaneous defects associated with SPPK and RASopathies share certain molecular faults. To identify intermediates responsible for executing the inhibition of ERK by DSG1, we conducted a yeast 2-hybrid screen. The screen revealed that Erbin (also known as ERBB2IP), a known ERK regulator, binds DSG1. Erbin silencing disrupted keratinocyte differentiation in culture, mimicking aspects of DSG1 deficiency. Furthermore, ERK inhibition and the induction of differentiation markers by DSG1 required both Erbin and DSG1 domains that participate in binding Erbin. Erbin blocks ERK signaling by interacting with and disrupting Ras-Raf scaffolds mediated by SHOC2, a protein genetically linked to the RASopathy, Noonan-like syndrome with loose anagen hair (NS/LAH). DSG1 overexpression enhanced this inhibitory function, increasing Erbin-SHOC2 interactions and decreasing Ras-SHOC2 interactions. Conversely, analysis of epidermis from DSG1-deficient patients with SPPK demonstrated increased Ras-SHOC2 colocalization and decreased Erbin-SHOC2 colocalization, offering a possible explanation for the observed epidermal defects. These findings suggest a mechanism by which DSG1 and Erbin cooperate to repress MAPK signaling and promote keratinocyte differentiation.
The Journal of Clinical Investigation