Molecular Basis for Dysregulated Activation of NKX2-5 in the Vascular Remodeling of Systemic Sclerosis.

Athina DritsoulaIoannis PapaioannouSandra G GuerraCarmen FonsecaJavier MartinAriane L HerrickDavid J AbrahamChristopher P DentonMarkella Ponticos

Published in: Arthritis & rheumatology (Hoboken, N.J.) (2018)

NKX2-5 is genetically associated with scleroderma, pulmonary hypertension, and fibrosis. Functional evidence revealed a regulatory mechanism that results in NKX2-5 transcriptional activation in PASMCs through the interaction of an upstream promoter and a novel downstream enhancer. This mechanism can act as a model for NKX2-5 activation in cardiovascular disease characterized by vascular remodeling.

Keyphrases

systemic sclerosis
interstitial lung disease
transcription factor
cardiovascular disease
pulmonary hypertension
gene expression
dna methylation
type diabetes
metabolic syndrome
coronary artery disease
liver fibrosis