Pathophysiological mechanisms of complications associated with propionic acidemia
Propionic acidemia (PA) is a genetic metabolic disorder caused by mutations in the
mitochondrial enzyme, propionyl-CoA carboxylase (PCC), which is responsible for …
mitochondrial enzyme, propionyl-CoA carboxylase (PCC), which is responsible for …
Cardiac complications of propionic and other inherited organic acidemias
Clinical observations and experimental studies have determined that systemic acid-base
disturbances can profoundly affect the heart. A wealth of information is available on the …
disturbances can profoundly affect the heart. A wealth of information is available on the …
Vericiguat alleviates ventricular remodeling and arrhythmias in mouse models of myocardial infarction via CaMKII signaling
T Chen, B Kong, W Shuai, Y Gong, J Zhang, H Huang - Life Sciences, 2023 - Elsevier
Aims Maladaptive ventricular remodeling is a major cause of ventricular arrhythmias
following myocardial infarction (MI) and adversely impacts the quality of life of affected …
following myocardial infarction (MI) and adversely impacts the quality of life of affected …
Pantothenate kinase activation relieves coenzyme A sequestration and improves mitochondrial function in mice with propionic acidemia
C Subramanian, MW Frank, R Tangallapally… - Science translational …, 2021 - science.org
Propionic acidemia (PA) is a rare autosomal-recessive metabolic disease that arises from
mutations in propionyl-CoA (C3-CoA) carboxylase. Reduced enzyme activity slows C3-CoA …
mutations in propionyl-CoA (C3-CoA) carboxylase. Reduced enzyme activity slows C3-CoA …
The attenuated hepatic clearance of propionate increases cardiac oxidative stress in propionic acidemia
Y Wang, S Zhu, W He, H Marchuk, E Richard… - Basic Research in …, 2024 - Springer
Propionic acidemia (PA), arising from PCCA or PCCB variants, manifests as life-threatening
cardiomyopathy and arrhythmias, with unclear pathophysiology. In this work, propionyl-CoA …
cardiomyopathy and arrhythmias, with unclear pathophysiology. In this work, propionyl-CoA …
Relief of CoA sequestration and restoration of mitochondrial function in a mouse model of propionic acidemia
C Subramanian, MW Frank… - Journal of Inherited …, 2023 - Wiley Online Library
Abstract Propionic acidemia (PA, OMIM 606054) is a devastating inborn error of metabolism
arising from mutations that reduce the activity of the mitochondrial enzyme propionyl‐CoA …
arising from mutations that reduce the activity of the mitochondrial enzyme propionyl‐CoA …
Metabolic perturbations mediated by propionyl-CoA accumulation in organs of mouse model of propionic acidemia
Propionic acidemia (PA) is an autosomal recessive metabolic disorder after gene encoding
propionyl-CoA carboxylase, Pcca or Pccb, is mutated. This genetic disorder could develop …
propionyl-CoA carboxylase, Pcca or Pccb, is mutated. This genetic disorder could develop …
Ca2+ mishandling in heart failure: Potential targets
A Val‐Blasco, M Gil‐Fernández, A Rueda… - Acta …, 2021 - Wiley Online Library
Ca2+ mishandling is a common feature in several cardiovascular diseases such as heart
failure (HF). In many cases, impairment of key players in intracellular Ca2+ homeostasis has …
failure (HF). In many cases, impairment of key players in intracellular Ca2+ homeostasis has …
Genetic and metabolic determinants of atrial fibrillation in a general population sample: the CHRIS study
Atrial fibrillation (AF) is a supraventricular arrhythmia deriving from uncoordinated electrical
activation with considerable associated morbidity and mortality. To expand the limited …
activation with considerable associated morbidity and mortality. To expand the limited …
Targeting the TWEAK–Fn14 pathway prevents dysfunction in cardiac calcium handling after acute kidney injury
J Poveda, L González‐Lafuente… - The Journal of …, 2023 - Wiley Online Library
Heart and kidney have a closely interrelated pathophysiology. Acute kidney injury (AKI) is
associated with significantly increased rates of cardiovascular events, a relationship defined …
associated with significantly increased rates of cardiovascular events, a relationship defined …