Soft substrates normalize nuclear morphology and prevent nuclear rupture in fibroblasts from a laminopathy patient with compound heterozygous LMNA mutations

Nucleus (United States)

Volumn 4, Issue 1, 2013, Pages 61-73

  Tamiello, Chiara ^a   Kamps, Miriam A F ^b,c   Van den Wijngaard, Arthur ^b   Verstraeten, Valerie L R M ^c   Baaijens, Frank P T ^a   Broers, Jos L V ^b   Bouten, Carlijn C V ^a  

^a EINDHOVEN UNIVERSITY OF TECHNOLOGY   (Netherlands)

^b MAASTRICHT UNIVERSITY   (Netherlands)

^c MAASTRICHT UNIVERSITY MEDICAL CENTER   (Netherlands)

Author keywords

Lamina; Laminopathies; Nuclear rupture; Nuclear shape alteration; Substrate stiffness

Indexed keywords

COLLAGEN TYPE 1; CYTOCHALASIN C; FLUORESCENT DYE; LAMIN A; LAMIN C; PHALLOIDIN;

ACTIN FILAMENT; ANTIBODY LABELING; ARTICLE; CELL COMPARTMENTALIZATION; CELL ISOLATION; CELL NUCLEUS MEMBRANE; CELL SPREADING; CHILD; CONFOCAL MICROSCOPY; CONTROLLED STUDY; FIBROBLAST; GENE MUTATION; GENETIC TRANSFECTION; HUMAN; HUMAN CELL; IMAGE ANALYSIS; IMAGING; IMMUNOFLUORESCENCE TEST; LAMINOPATHY; MALE; MEMBRANE RUPTURE; PRESCHOOL CHILD; SKIN BIOPSY; CARDIOMYOPATHY; CELL LINE; CELL NUCLEUS; CHEMISTRY; CYTOLOGY; GENETICS; HETEROZYGOTE; LAMINA; LAMINOPATHIES; METABOLISM; MUTATION; NUCLEAR RUPTURE; NUCLEAR SHAPE; NUCLEAR SHAPE ALTERATION; PATHOLOGY; PHYSIOLOGY; PROGERIA; SUBSTRATE STIFFNESS;

LAMINA; LAMINOPATHIES; NUCLEAR RUPTURE; NUCLEAR SHAPE ALTERATION; SUBSTRATE STIFFNESS;

ACTIN CYTOSKELETON; CARDIOMYOPATHIES; CELL LINE; CELL NUCLEUS; CELL NUCLEUS SHAPE; FIBROBLASTS; HETEROZYGOTE; HUMANS; LAMIN TYPE A; MUTATION; PROGERIA;

EID: 84873533631     PISSN: 19491034     EISSN: 19491042     Source Type: Journal    
DOI: 10.4161/nucl.23388     Document Type: Article

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