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The Cardiac Sarcomere and Cell Cycle
Purpose of ReviewThe lack of adult human cardiomyocyte proliferative capacity impairs cardiac regeneration such as after myocardial injury. The...
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Intervertebral disc degeneration is rescued by TGFβ/BMP signaling modulation in an ex vivo filamin B mouse model
Spondylocarpotarsal syndrome (SCT) is a rare musculoskeletal disorder characterized by short stature and vertebral, carpal, and tarsal fusions...
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Cytopenia in autosomal dominant polycystic kidney disease (ADPKD): merely an association or a disease-related feature with prognostic implications?
Autosomal dominant polycystic kidney disease (ADPKD) is associated with distinct cytopenias in observational studies; the most consistent and...
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Dilated cardiomyopathy in the era of precision medicine: latest concepts and developments
Dilated cardiomyopathy (DCM) is an umbrella term entailing a wide variety of genetic and non-genetic etiologies, leading to left ventricular systolic...
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Rare clinical phenotype of filaminopathy presenting as restrictive cardiomyopathy and myopathy in childhood
BackgroundFLNC is one of the few genes associated with all types of cardiomyopathies, but it also underlies neuromuscular phenotype. The combination...
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Role of biophysics and mechanobiology in podocyte physiology
Podocytes form the backbone of the glomerular filtration barrier and are exposed to various mechanical forces throughout the lifetime of an...
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High fat diet causes distinct aberrations in the testicular proteome
Diet has important effects on normal physiology and the potential deleterious effects of high fat diets and obesity on male reproductive health are...
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Spondylocarpotarsal synostosis syndrome due to a novel loss of function FLNB variant: a case report
BackgroundLoss of function or gain of function variants of Filamin B ( FLNB) cause recessive or dominant skeletal disorders respectively....
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Emerging concepts in arrhythmogenic dilated cardiomyopathy
Dilated cardiomyopathy (DCM) represents one of the primary cardiomyopathies and may lead to heart failure and sudden death. Until recently,...
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The identification of nuclear αvβ3 integrin in ovarian cancer: non-paradigmal localization with cancer promoting actions
Nuclear translocation of transmembrane proteins was reported in high-grade serous ovarian cancer (HGSOC), a highly aggressive gynecological...
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Identification of Gene Mutations in Primary Pediatric Cardiomyopathy by Whole Exome Sequencing
Pediatric primary cardiomyopathy is rare but serious, having high mortality; hypertrophic and dilated types are the most common. Its etiology has...
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A case report: a heterozygous deletion (2791_2805 del) in exon 18 of the filamin C gene causing filamin C-related myofibrillar myopathies in a Chinese family
BackgroundFilamin C-related myofibrillar myopathies (MFM) are progressive skeletal myopathies with an autosomal dominant inheritance pattern. The...
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Congenital emphysematous lung disease associated with a novel Filamin A mutation. Case report and literature review
BackgroundProgressive lung involvement in Filamin A (FLNA)-related cerebral periventricular nodular heterotopia (PVNH) has been reported in a limited...
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Talin and Kindlin as Integrin-Activating Proteins: Focus on the Heart
Integrin receptors enable cells to sense and respond to their chemical and physical environment. As a class of membrane receptors, they provide a...
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Molecular mechanisms of arrhythmogenic cardiomyopathy
Arrhythmogenic cardiomyopathy is a genetic disorder characterized by the risk of life-threatening arrhythmias, myocardial dysfunction and fibrofatty...
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Overexpression of filamin c in chronic intermittent hypoxia-induced cardiomyocyte apoptosis is a potential cardioprotective target for obstructive sleep apnea
PurposeChronic intermittent hypoxia (CIH) is key pathological mechanism of obstructive sleep apnea (OSA), which induced cardiac dysfunction. Filamin...
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Silencing Filamin A Inhibits the Invasion and Migration of Breast Cancer Cells by Up-regulating 14-3-3σ
Filamin A and 14-3-3-σ are closely associated with the development of breast cancer. However, the exact relationship between them is still unknown....
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Targeting filamin B induces tumor growth and metastasis via enhanced activity of matrix metalloproteinase-9 and secretion of VEGF-A
Filamins regulate cell locomotion and associate with diverse signaling molecules. We have recently found that targeting filamin A (FLNA) reduces...
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Heat-shock factor 2 is a suppressor of prostate cancer invasion
Heat-shock factors (HSFs) are key transcriptional regulators in cell survival. Although HSF1 has been identified as a driver of carcinogenesis, HSF2...
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Filamin C-related myopathies: pathology and mechanisms
The term filaminopathy was introduced after a truncating mutation in the dimerization domain of filamin C (FLNc) was shown to be responsible for a...
