title: Fibrinogen Sapporo: dysfibrinogenemia characterized by the replacement of A alpha arginine-16 by histidine resulting in the delayed release of fibrinopeptide A by thrombin.
abstract: Congenital dysfibrinogenemia was found in a 60-year-old asymptomatic female and her daughter. Purified fibrinogen derived from the propositus, apparently a heterozygote for the abnormality, characteristically showed delayed but complete release of fibrinopeptide A upon digestion with thrombin but its defective release by Ancrod, a snake venom enzyme, from half of her fibrinogen molecules. This congenital dysfibrinogenemia with an A alpha arginine (Arg) to histidine (His) substitution was tentatively designated as fibrinogen Sapporo. Although this type of abnormal fibrinogen had been identified among Caucasians, no such cases have so far been reported in Japan.
authors.forename: S
authors.initials: S
authors.lastname: Asakura
authors.forename: S
authors.initials: S
authors.lastname: Terukina
authors.forename: K
authors.initials: K
authors.lastname: Yamazumi
authors.forename: M
authors.initials: M
authors.lastname: Matsuda
authors.forename: H
authors.initials: H
authors.lastname: Murayama
authors.forename: A
authors.initials: A
authors.lastname: Higuchi
authors.forename: M
authors.initials: M
authors.lastname: Musashi
authors.forename: K
authors.initials: K
authors.lastname: Sakurada
authors.forename: T
authors.initials: T
authors.lastname: Miyazaki
mesh_terms: D005260:Female; D005340:Fibrinogen / Q000378:metabolism*; D015241:Fibrinogens, Abnormal / Q000302:isolation & purification* / Q000378:metabolism; D005344:Fibrinopeptide A / Q000378:metabolism*; D006801:Humans; D007564:Japan; D008875:Middle Aged; D013917:Thrombin / Q000378:metabolism*; D013997:Time Factors
chemical_list: D015241:Fibrinogens, Abnormal; C053388:fibrinogen Giessen I; D005344:Fibrinopeptide A; D005340:Fibrinogen; D013917:Thrombin