The effect of thyroxine on ATP-sensitive potassium channels and cardiolipin synthesis [PDF]
, 2015 甲亢性心脏病（HyperthyroidHeartDisease,HHD）是甲状腺功能亢进（Hyperthyrodism）的严重并发症，严重危害患者的生命健康，但其机制尚未完全阐明。心磷脂（Cardiolipin，CL）是主要位于线粒体内膜的特殊磷脂，在维持正常的线粒体功能中具有至关重要的作用。本课题组前期研究表明甲亢小鼠心脏中心磷脂含量升高，并且心磷脂参与甲亢性心脏病的病理过程。此外，文献资料显示甲亢会引起心磷脂侧链的C18:2的丢失，同时引起侧链中多不饱和脂肪酸含量的增多 ...
侯琦
core
Genetics of human and canine dilated cardiomyopathy [PDF]
, 2015 Cardiovascular disease is a leading cause of death in both humans and dogs. Dilated cardiomyopathy (DCM) accounts for a large number of these cases, reported to be the third most common form of cardiac disease in humans and the second most common in dogs.
Cobb, Malcolm +5 more
core +5 more sources
Characterization of a Transgenic Short Hairpin RNA-Induced Murine Model of Tafazzin Deficiency [PDF]
Human Gene Therapy, 2011 Barth's syndrome (BTHS) is an X-linked mitochondrial disease that is due to a mutation in the Tafazzin (TAZ) gene. Based on sequence homology, TAZ has been characterized as an acyltransferase involved in the metabolism of cardiolipin (CL), a unique phospholipid almost exclusively located in the mitochondrial inner membrane.
Meghan S, Soustek +6 more
openaire +2 more sources
Frontiers in Genetics, 2016 Mutations in the gene encoding the enzyme tafazzin, TAZ, cause Barth syndrome (BTHS). Individuals with this X-linked multisystem disorder present cardiomyopathy (often dilated), skeletal muscle weakness, neutropenia, growth retardation and 3 ...
Ana eSaric +5 more
doaj +1 more source
Cardiac metabolic pathways affected in the mouse model of barth syndrome. [PDF]
PLoS ONE, 2015 Cardiolipin (CL) is a mitochondrial phospholipid essential for electron transport chain (ETC) integrity. CL-deficiency in humans is caused by mutations in the tafazzin (Taz) gene and results in a multisystem pediatric disorder, Barth syndrome (BTHS).
Yan Huang +9 more
doaj +1 more source
A predictive model for canine dilated cardiomyopathy: a meta-analysis of Doberman Pinscher data [PDF]
, 2015 Dilated cardiomyopathy is a prevalent and often fatal disease in humans and dogs. Indeed dilated cardiomyopathy is the third most common form of cardiac disease in humans, reported to a ect approximately 36 individuals per 100,000 individuals.
Bland +27 more
core +6 more sources
Correction of human phospholamban R14del mutation associated with cardiomyopathy using targeted nucleases and combination therapy [PDF]
, 2015 published_or_final_versio
Asselbergs, FW +23 more
core +1 more source
Kidney Fibrosis In Vitro and In Vivo Models: Path Toward Physiologically Relevant Humanized Models
Advanced Healthcare Materials, Volume 14, Issue 9, April 4, 2025.This review discusses kidney diseases with a focus on kidney fibrosis and the models currently used. It highlights the limitations of animal and 2D in vitro models in replicating human kidney anatomy and physiology. Consequently, there is a significant need for humanized 3D in vitro models, such as 3D cell aggregates, membranes and hydrogels, on‐chip ...
Gabriele Addario +2 more
wiley +1 more source
G-Protein coupled receptor signalling in pluripotent stem cell-derived cardiovascular cells: Implications for disease modelling [PDF]
, 2015 Human pluripotent stem cell derivatives show promise as an in vitro platform to study a range of human cardiovascular diseases. A better understanding of the biology of stem cells and their cardiovascular derivatives will help to understand the strengths
Dolatshad, NF +4 more
core +1 more source
Advanced Science, Volume 12, Issue 15, April 17, 2025.Targeting USP1 is identified as a strategy that significantly enhances type I IFN‐dependent innate immune response within the TME following radiotherapy. Mechanistically, USP1 inhibition facilitates STING trafficking from ER to Golgi by preserving K27‐linked oligo‐ubiquitination of SAR1A, thereby facilitating efficient activation of the dsDNA‐cGAS ...
Weilin Zhou +15 more
wiley +1 more source