0000000000053726

AUTHOR

Attila Aszódi

showing 3 related works from this author

Profilin 1 is required for abscission during late cytokinesis of chondrocytes

2009

Profilins are key factors for dynamic rearrangements of the actin cytoskeleton. However, the functions of profilins in differentiated mammalian cells are uncertain because profilin deficiency is early embryonic lethal for higher eukaryotes. To examine profilin function in chondrocytes, we disrupted the profilin 1 gene in cartilage (Col2pfn1). Homozygous Col2pfn1 mice develop progressive chondrodysplasia caused by disorganization of the growth plate and defective chondrocyte cytokinesis, indicated by the appearance of binucleated cells. Surprisingly, Col2pfn1 chondrocytes assemble and contract actomyosin rings normally during cell division; however, they display defects during late cytokines…

Cell divisionMice Transgenicmacromolecular substancesBiologyMyosinsOsteochondrodysplasiasGeneral Biochemistry Genetics and Molecular BiologyChondrocyteArticleBone and BonesMiceProfilinsChondrocytesMyosinmedicineAnimalsMolecular BiologyActinCytokinesisGeneral Immunology and MicrobiologyGeneral NeuroscienceActin cytoskeletonActinsCell biologymedicine.anatomical_structureCartilageProfilinGene Targetingbiology.proteinLamellipodiumCytokinesis
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Perlecan Maintains the Integrity of Cartilage and Some Basement Membranes

1999

Perlecan is a heparan sulfate proteoglycan that is expressed in all basement membranes (BMs), in cartilage, and several other mesenchymal tissues during development. Perlecan binds growth factors and interacts with various extracellular matrix proteins and cell adhesion molecules. Homozygous mice with a null mutation in the perlecan gene exhibit normal formation of BMs. However, BMs deteriorate in regions with increased mechanical stress such as the contracting myocardium and the expanding brain vesicles showing that perlecan is crucial for maintaining BM integrity. As a consequence, small clefts are formed in the cardiac muscle leading to blood leakage into the pericardial cavity and an ar…

Heart Defects Congenitalcardiac muscleMesenchymeSchwartz–Jampel syndromeRestriction MappingPerlecanBasement MembraneExtracellular matrixMiceMice CongenicchondrodysplasiaCalcification PhysiologicexencephalyLamininmedicineAnimalsNeural Tube DefectsCells CulturedBasement membranebiologyCartilageOssification HeterotopicHomozygoteCell Biologymedicine.diseaseMice Mutant StrainsBasement membrane assemblyCell biologyperlecanMutagenesis Insertionalmedicine.anatomical_structureCartilageBiochemistryGene Targetingbiology.proteinOriginal ArticleGenes LethalProteoglycansCollagenHeparitin SulfateExostoses Multiple HereditaryHeparan Sulfate ProteoglycansThe Journal of Cell Biology
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Perlecan is critical for heart stability

2008

Aims Perlecan is a heparansulfate proteoglycan found in basement membranes, cartilage, and several mesenchymal tissues that form during development, tumour growth, and tissue repair. Loss-of-function mutations in the perlecan gene in mice are associated with embryonic lethality caused primarily by cardiac abnormalities probably due to hemopericards. The aim of the present study was to investigate the mechanism underlying the early embryonic lethality and the pathophysiological relevance of perlecan for heart function. Methods and results Perlecan-deficient murine embryonic stem cells were used to investigate the myofibrillar network and the electrophysiological properties of single cardiomy…

Patch-Clamp TechniquesPhysiologyMyocardial InfarctionMice TransgenicCell CommunicationPerlecanSarcomereBasement MembraneVentricular Function LeftAdherens junctionExtracellular matrixMicePhysiology (medical)medicineAnimalsMyocytes CardiacCells CulturedEmbryonic Stem CellsBasement membranebiologyCartilageCell DifferentiationHeartAnatomyEmbryonic stem cellCell biologyMice Inbred C57BLcarbohydrates (lipids)Disease Models Animalmedicine.anatomical_structurebiology.proteinFemaleCardiology and Cardiovascular MedicineMyofibrilHeparan Sulfate ProteoglycansCardiovascular Research
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