Clinical, radiologic, pathologic, and molecular characteristics of long-term survivors of diffuse intrinsic pontine glioma (DIPG): a collaborative report from the International and European Society for Pediatric Oncology DIPG registries [PDF]
, 2018 Purpose Diffuse intrinsic pontine glioma (DIPG) is a brainstem malignancy with a median survival of < 1 year. The International and European Society for Pediatric Oncology DIPG Registries collaborated to compare clinical, radiologic, and ...
Antonelli, Manila +73 more
core +7 more sources
MicroRNA-483 amelioration of experimental pulmonary hypertension. [PDF]
, 2020 Endothelial dysfunction is critically involved in the pathogenesis of pulmonary arterial hypertension (PAH) and that exogenously administered microRNA may be of therapeutic benefit.
Bai, Liang +22 more
core +1 more source
ACVR1: A Novel Therapeutic Target to Treat Anemia in Myelofibrosis
Cancers, 2023 Activin receptor type I (ACVR1) is a transmembrane kinase receptor belonging to bone morphogenic protein receptors (BMPs). ACVR1 plays an important role in hematopoiesis and anemia via the BMP6/ACVR1/SMAD pathway, which regulates expression of hepcidin, the master regulator of iron homeostasis.
Andrea Duminuco +5 more
openaire +3 more sources
Fibrodysplasia ossificans progressiva: current concepts from bench to bedside
Disease Models & Mechanisms, 2020 Heterotopic ossification (HO) is a disorder characterised by the formation of ectopic bone in soft tissue. Acquired HO typically occurs in response to trauma and is relatively common, yet its aetiology remains poorly understood.
Arun-Kumar Kaliya-Perumal +2 more
doaj +1 more source
ACVR1 Mutations in DIPG: Lessons Learned from FOP [PDF]
Cancer Research, 2014 Abstract Whole-genome sequencing studies have recently identified a quarter of cases of the rare childhood brainstem tumor diffuse intrinsic pontine glioma to harbor somatic mutations in ACVR1. This gene encodes the type I bone morphogenic protein receptor ALK2, with the residues affected identical to those that, when mutated in the ...
Taylor, K +3 more
openaire +3 more sources
Activation of AcvR1-Mediated Signaling Results in Semilunar Valve Defects
Journal of Cardiovascular Development and Disease, 2022 Calcific aortic valve disease (CAVD) is a common cardiac defect, particularly in the aging population. While several risk factors, such as bi-leaflet valve structure and old age, have been identified in CAVD pathogenesis, molecular mechanisms resulting in this condition are still under active investigation.
Shabber Syed +8 more
openaire +3 more sources
Activin and TGFβ use diverging mitogenic signaling in advanced colon cancer. [PDF]
, 2015 BackgroundUnderstanding cell signaling pathways that contribute to metastatic colon cancer is critical to risk stratification in the era of personalized therapeutics.
Akagi, Naomi +9 more
core +2 more sources
Function of Bmpr1a in ES cell differentiation and cell competition [PDF]
, 2010 Bone morphogenetic protein (BMP) 4 signalling via BMPR1A is required for the maintenance of the epiblast in the early embryo, and for self-renewal of pluripotent mouse embryonic stem (ES) cells by inhibiting neural differentiation.
Sancho, Maria Margarida Gouveia +1 more
core +1 more source
eLife, 2020 Activin A functions in BMP signaling in two ways: it either engages ACVR1B to activate Smad2/3 signaling or binds ACVR1 to form a non-signaling complex (NSC).
Senem Aykul +19 more
doaj +1 more source
Modeling the ACVR1R206H mutation in human skeletal muscle stem cells
eLife, 2021 Abnormalities in skeletal muscle repair can lead to poor function and complications such as scarring or heterotopic ossification (HO). Here, we use fibrodysplasia ossificans progressiva (FOP), a disease of progressive HO caused by ACVR1R206H (Activin ...
Emilie Barruet +7 more
doaj +1 more source