Differential effects of the loss of intrachain- versus interchain-disulfide bonds in the cystine-knot domain of von Willebrand factor on the clinical phenotype of von Willebrand disease

Thrombosis and Haemostasis

Volumn 96, Issue 6, 2006, Pages 717-724

  Tjernberg, Pernilla ^a   Vos, Hans L ^a   Spaargaren van Riel, Caroline C ^a   Luken, Brenda M ^b   Voorberg, Jan ^b   Bertina, Rogier M ^a   Eikenboom, Jeroen C J ^a  

^a LEIDEN UNIVERSITY MEDICAL CENTER   (Netherlands)

^b ACADEMIC MEDICAL CENTER   (Netherlands)

Author keywords

ADAMTS13; Cysteine; Dimerization; Disulfide bond; Multimerization

Indexed keywords

CYSTEINE; CYSTINE; POLYMER; RECOMBINANT PROTEIN; VON WILLEBRAND FACTOR; VON WILLEBRAND FACTOR CLEAVING PROTEINASE;

AMINO TERMINAL SEQUENCE; ARTICLE; CARBOXY TERMINAL SEQUENCE; CLINICAL ARTICLE; CONTROLLED STUDY; CYSTINE KNOT MOTIF; DIMERIZATION; DISULFIDE BOND; ELECTROPHORETIC MOBILITY; FEMALE; GENE CONSTRUCT; GENE EXPRESSION; GENETIC TRANSFECTION; HETEROZYGOSITY; HUMAN; HUMAN CELL; MALE; MOLECULAR WEIGHT; MUTATIONAL ANALYSIS; NUCLEOTIDE SEQUENCE; PHENOTYPIC VARIATION; POLYACRYLAMIDE GEL ELECTROPHORESIS; POLYMERIZATION; PRIORITY JOURNAL; PROTEIN DEGRADATION; QUALITATIVE ANALYSIS; QUANTITATIVE ANALYSIS; SCREENING TEST; VON WILLEBRAND DISEASE;

ADAM PROTEINS; BLOOD COAGULATION; CELL LINE; CYSTEINE; CYSTINE KNOT MOTIFS; DIMERIZATION; DISULFIDES; DNA MUTATIONAL ANALYSIS; ELECTROPHORESIS, POLYACRYLAMIDE GEL; FEMALE; HUMANS; MALE; MUTATION, MISSENSE; PEDIGREE; PHENOTYPE; PROTEIN CONFORMATION; PROTEIN PROCESSING, POST-TRANSLATIONAL; TRANSFECTION; VON WILLEBRAND DISEASE; VON WILLEBRAND FACTOR;

EID: 33845369823     PISSN: 03406245     EISSN: None     Source Type: Journal    
DOI: 10.1160/TH06-08-0460     Document Type: Article

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