Neurofibromatosis type 1 and pulmonary arterial hypertension: A case report

Santos, M; Pereira, Andreia

doi:10.1016/j.repc.2019.05.018

Cited by 2 publications

(1 citation statement)

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“…Human patients with pathogenic variants in the NF1 gene have been found to experience pulmonary arterial remodeling and PH. However, the mechanism behind this association remains unknown and requires further investigation [64]. LGALS3BP belongs to the scavenger receptor cysteine-rich domain family and has been found to be involved in immunity and inflammation [65].…”

Section: Plos Onementioning

confidence: 99%

Proteomic analysis of pulmonary arteries and lung tissues from dogs affected with pulmonary hypertension secondary to degenerative mitral valve disease

Sakarin,

Rungsipipat,

Roytrakul

et al. 2024

PLoS ONE

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In dogs with degenerative mitral valve disease (DMVD), pulmonary hypertension (PH) is a common complication characterized by abnormally elevated pulmonary arterial pressure (PAP). Pulmonary arterial remodeling is the histopathological changes of pulmonary artery that has been recognized in PH. The underlying mechanisms that cause this arterial remodeling are poorly understood. This study aimed to perform shotgun proteomics to investigate changes in protein expression in pulmonary arteries and lung tissues of DMVD dogs with PH compared to normal control dogs and DMVD dogs without PH. Tissue samples were collected from the carcasses of 22 small-sized breed dogs and divided into three groups: control (n = 7), DMVD (n = 7) and DMVD+PH groups (n = 8). Differentially expressed proteins were identified, and top three upregulated and downregulated proteins in the pulmonary arteries of DMVD dogs with PH including SIK family kinase 3 (SIK3), Collagen type I alpha 1 chain (COL1A1), Transforming growth factor alpha (TGF-α), Apoptosis associated tyrosine kinase (AATYK), Hepatocyte growth factor activator (HGFA) and Tyrosine-protein phosphatase non-receptor type 13 (PTPN13) were chosen. Results showed that some of the identified proteins may play a role in the pathogenesis of pulmonary arterial remodeling. This study concluded shotgun proteomics has potential as a tool for exploring candidate proteins associated with the pathogenesis of PH secondary to DMVD in dogs.

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Section: Plos Onementioning

confidence: 99%