title: Primary structure of the factor VIII binding domain of human, porcine and rabbit von Willebrand factor.
abstract: von Willebrand factor (vWF) binds to Factor VIII (FVIII) and the FVIII binding domain has been localized to the amino-terminal of the vWF subunit. The DNA sequence coding for part of the vWF precursor (provWF) including the FVIII binding domain has been compared in man, pig and rabbit. The sequenced fragment corresponds to nucleotides 2416 to 2886 of the human vWF cDNA and encodes for the last 41 amino acids of the propolypeptide, the cleavage site and the first 116 amino acids of the mature vWF subunit. The homology of the three deduced amino acids sequences is remarkable: 88% between porcine and human and 87% between rabbit and human sequences. Four contiguous amino acids are lacking in the rabbit propolypeptide (-10 to -7) when compared to the human and porcine sequences. The cleavage site of the propolypeptide is conserved in the three species as well as amino acids where mutations in the human gene lead to a binding defect of vWF to FVIII. The Asn-94 N-glycosylation site is present in the human and rabbit sequences but absent in the pig sequence.
authors.affiliation: INSERM U143, Hôpital de Bicêtre, France.
authors.forename: J M
authors.initials: JM
authors.lastname: Lavergne
authors.forename: Y C
authors.initials: YC
authors.lastname: Piao
authors.forename: V
authors.initials: V
authors.lastname: Ferreira
authors.forename: D
authors.initials: D
authors.lastname: Kerbiriou-Nabias
authors.forename: B R
authors.initials: BR
authors.lastname: Bahnak
authors.forename: D
authors.initials: D
authors.lastname: Meyer
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D001483:Base Sequence; D001665:Binding Sites; D005169:Factor VIII / Q000378:metabolism*; D006031:Glycosylation; D006801:Humans; D008969:Molecular Sequence Data; D010446:Peptide Fragments / Q000235:genetics*; D016133:Polymerase Chain Reaction; D011498:Protein Precursors / Q000235:genetics*; D011499:Protein Processing, Post-Translational; D017433:Protein Structure, Secondary; D011817:Rabbits; D017422:Sequence Analysis, DNA; D017386:Sequence Homology, Amino Acid; D013552:Swine; D014841:von Willebrand Factor / Q000235:genetics* / Q000378:metabolism
chemical_list: D010446:Peptide Fragments; D011498:Protein Precursors; D014841:von Willebrand Factor; D005169:Factor VIII