New ProteomeXchange dataset PXD053691

[ProteomeCentral Agent]

Dear ProteomeXchange subscriber, a new ProteomeXchange dataset is being announced. To see more information, click here:

https://proteomecentral.proteomexchange.org/dataset/PXD053691

Summary of dataset

Status: new
Identifier: PXD053691
HostingRepository: PRIDE
Species: Homo sapiens
Title: Interleukin-8 mediated cellular crosstalk drives hypertrophic cardiomyopathy in LZTR1 2 deficiency
Submitter: Christof Lenz
LabHead: Christof Lenz
Description: LZTR1 deficiency causes Noonan syndrome (NS) and is associated with the manifestation of severe and early-onset hypertrophic cardiomyopathy (HCM). Although paracrine communication is thought to play a critical role in cardiac pathology, little is known about the underlying pathomechanism leading to the development of HCM and cardiac fibrosis in NS. In the present study, we used 2D and 3D iPSC models with LZTR1 deficiency to dissect the impact of non-cardiomyocytes in the development of NS-associated HCM and uncovered a cytokine mediated intercellular crosstalk, predominantly activated in the disease state, as a major driver of cardiac hypertrophy and cardiac fibrosis. We showed that cardiac fibroblast-specific secretion of interleukin-8 induces a fibrosis-related signature and potentiates cardiomyocyte 45 hypertrophy. Importantly, inhibition of IL-8 signaling reversed the pathological effects in 46 cardiac fibroblasts and cardiomyocytes and may serve as a novel therapeut
ic option for the 47 treatment of NS-associated HCM.

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