title: Characterization of a thrombopathy (type delta storage pool disease) affecting a pig colony.
abstract: An autosomal recessive thrombopathy in pigs is described and characterized functionally, morphologically and biochemically. The affected pigs have a severe bleeding diathesis and a markedly prolonged bleeding time but normal plasma and platelet von Willebrand factor (vWF) levels. Electron micrographs and fluorescence microscopy with mepacrine reveal reduced numbers of dense granules in platelets as compared to normals. This thrombopathy is a pure delta storage pool disease (SPD), as evidenced: a) biochemically by platelet serotonin content and metabolism and by comparative ATP/ADP content and secretion; b) functionally by reduced aggregability to low concentrations of convulxin and collagen but normal aggregability to other agents and normal synthesis of thromboxane B2. The affection was first discovered in a colony of von Willebrand's disease (vWD) pigs, but is biologically and genetically distinct. It is possible to completely separate the SPD from the vWD, although originally animals could be affected by both vWD and SPD. Normal plasma and platelet alpha granule content of vWF are found in diseased animals. An intermediate disorder is also detected in animals not severely affected, which may represent the heterozygous state.
authors.affiliation: INSERM U 353, Hôpital Saint-Louis, Paris, France.
authors.forename: H
authors.initials: H
authors.lastname: Radvanyi-Hofmann
authors.forename: J
authors.initials: J
authors.lastname: Roussi
authors.forename: J M
authors.initials: JM
authors.lastname: Launay
authors.forename: M
authors.initials: M
authors.lastname: Bonneau
authors.forename: M C
authors.initials: MC
authors.lastname: Prevost
authors.forename: B
authors.initials: B
authors.lastname: Vargaftig
authors.forename: L
authors.initials: L
authors.lastname: Drouet
mesh_terms: D000121:Acetylserotonin O-Methyltransferase / Q000097:blood; D000227:Adenine Nucleotides / Q000097:blood; D000818:Animals; D001191:Arylamine N-Acetyltransferase / Q000097:blood; D001792:Blood Platelets / Q000737:chemistry / Q000648:ultrastructure; D003594:Cytoplasmic Granules / Q000737:chemistry; D005808:Genes, Recessive; D008854:Microscopy, Electron; D010974:Platelet Aggregation; D010981:Platelet Storage Pool Deficiency / Q000097:blood / Q000150:complications / Q000235:genetics / Q000662:veterinary*; D012701:Serotonin / Q000097:blood; D013552:Swine / Q000097:blood / Q000235:genetics; D013553:Swine Diseases / Q000097:blood / Q000235:genetics*; D013929:Thromboxane B2 / Q000097:blood; D014842:von Willebrand Diseases / Q000150:complications; D014841:von Willebrand Factor / Q000032:analysis
chemical_list: D000227:Adenine Nucleotides; D014841:von Willebrand Factor; D012701:Serotonin; D013929:Thromboxane B2; D000121:Acetylserotonin O-Methyltransferase; D001191:Arylamine N-Acetyltransferase