Classical and neonatal Marfan syndrome mutations in fibrillin-1 cause differential protease susceptibilities and protein function

Journal of Biological Chemistry

Volumn 286, Issue 37, 2011, Pages 32810-32823

  Kirschner, Ryan ^a   Hubmacher, Dirk ^b   Iyengar, Garud ^b   Kaur, Jasvir ^b   Fagotto Kaufmann, Christine ^b   Brömme, Dieter ^c   Bartels, Rainer ^d   Reinhardt, Dieter P ^a,b  

^a MCGILL UNIVERSITY   (Canada)

^b MCGILL UNIVERSITY   (Canada)

^c UNIVERSITY OF BRITISH COLUMBIA   (Canada)

^d Tuberculosis Center Borstel   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

BIOCHEMISTRY; CIRCULAR DICHROISM SPECTROSCOPY; DICHROISM; ENZYMES; GEL PERMEATION CHROMATOGRAPHY; POLYSACCHARIDES; SULFUR COMPOUNDS;

EXTRACELLULAR MATRICES; GEL-FILTRATION CHROMATOGRAPHY; MATRIX METALLOPROTEINASES; MICROFIBRIL ASSEMBLY; PROTEOLYTIC CLEAVAGE; PROTEOLYTIC SUSCEPTIBILITY; SECONDARY STRUCTURES; WILD-TYPE PROTEINS;

BLOOD VESSELS;

CATHEPSIN; CATHEPSIN K; CATHEPSIN V; FIBRILLIN 1; HEPARAN SULFATE; HEPARIN; MATRIX METALLOPROTEINASE; PLASMIN; THROMBIN;

ARTICLE; CIRCULAR DICHROISM; CONFORMATIONAL TRANSITION; CONTROLLED STUDY; DISEASE ACTIVITY; DISEASE COURSE; FIBER; GEL FILTRATION CHROMATOGRAPHY; GENE MUTATION; GENETIC SUSCEPTIBILITY; HUMAN; HUMAN CELL; MARFAN SYNDROME; MOLECULAR DYNAMICS; PATHOGENESIS; POINT MUTATION; PRIORITY JOURNAL; PROTEIN CLEAVAGE; PROTEIN DEGRADATION; PROTEIN EXPRESSION; PROTEIN FUNCTION; PROTEIN INTERACTION; PROTEIN SECONDARY STRUCTURE; PROTEIN STRUCTURE; SENSITIVITY ANALYSIS; THERMOSTABILITY;

EID: 80052699885     PISSN: 00219258     EISSN: 1083351X     Source Type: Journal    
DOI: 10.1074/jbc.M111.221804     Document Type: Article

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