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184,185,203 It is important to note that because most PK-deficient patients are compound heterozygous for two different (missense) mutations, up to seven different tetrameric forms of PK may be present in such patients, each with distinct structural and kinetic properties

10.1111/jnc.13248 86 ManciasJ

med.) of Marja-Lisa Berthold at RWTH Aachen University, Faculty of Medicine, Germany

these bioactive molecules act on multiple targetsincluding EMT-related transcription factors and key signaling cascadesto effectively block epithelial-mesenchymal conversion, suppress myofibroblast activation and regulate aberrant extracellular-matrix (ECM) remodeling, thereby markedly curbing EMT progression and fibrotic disease ( Triptolide (TP), an active diterpenoid extracted from Tripterygium wilfordii Hook F, possesses anti-inflammatory, antiproliferative and immunomodulatory properties, and has been widely investigated in experimental idiopathic pulmonary fibrosis (IPF) models in recent years (102)