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Defective gamma-glutamyl carboxylase activity and bleeding in Rambouillet sheep.
Vet Pathol 2006; 43(5):726-32VP

Abstract

A flock of Rambouillet sheep was examined because of increased lamb mortality caused by ineffective hemostasis at parturition. Neonatal-affected lambs presented with inadequate hemostasis at the umbilicus, pale mucus membranes, and markedly prolonged activated clotting time. Affected lambs had consistently prolonged 1-stage prothrombin times and activated partial thromboplastin times that supported a defect in the common pathway or defects in both the intrinsic and extrinsic pathway of the coagulation cascade. Decreased activity of vitamin K-dependent procoagulant factors II, VII, IX, and X in male and female lambs suggested either a defect of the hepatic enzyme gamma-glutamyl carboxylase, or vitamin K(1) 2,3 epoxide reductase. Affected lamb hepatic gamma-glutamyl carboxylase activity was markedly decreased compared with that of age- and sex-matched control lambs, while vitamin K(1) 2,3 epoxide reductase and glucose-6-phosphatase activities were similar between an affected and normal lamb. Subcutaneous vitamin K(1) supplementation did not increase vitamin K-dependent procoagulant factor activities in 3 lambs administered vitamin K(1) daily. These data confirm defective gamma-glutamyl carboxylase activity as the cause of impaired coagulation of sheep in this flock. This flock represents the only viable animal model of hereditarily defective gamma-glutamyl carboxylase activity.

Authors+Show Affiliations

Department of Microbiology, Immunology, and Pathology, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO, USA. jeremy-johnson@idexx.com

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

16966451

Citation

Johnson, J S., et al. "Defective Gamma-glutamyl Carboxylase Activity and Bleeding in Rambouillet Sheep." Veterinary Pathology, vol. 43, no. 5, 2006, pp. 726-32.
Johnson JS, Soute BA, Olver CS, et al. Defective gamma-glutamyl carboxylase activity and bleeding in Rambouillet sheep. Vet Pathol. 2006;43(5):726-32.
Johnson, J. S., Soute, B. A., Olver, C. S., & Baker, D. C. (2006). Defective gamma-glutamyl carboxylase activity and bleeding in Rambouillet sheep. Veterinary Pathology, 43(5), pp. 726-32.
Johnson JS, et al. Defective Gamma-glutamyl Carboxylase Activity and Bleeding in Rambouillet Sheep. Vet Pathol. 2006;43(5):726-32. PubMed PMID: 16966451.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Defective gamma-glutamyl carboxylase activity and bleeding in Rambouillet sheep. AU - Johnson,J S, AU - Soute,B A, AU - Olver,C S, AU - Baker,D C, PY - 2006/9/13/pubmed PY - 2006/11/9/medline PY - 2006/9/13/entrez SP - 726 EP - 32 JF - Veterinary pathology JO - Vet. Pathol. VL - 43 IS - 5 N2 - A flock of Rambouillet sheep was examined because of increased lamb mortality caused by ineffective hemostasis at parturition. Neonatal-affected lambs presented with inadequate hemostasis at the umbilicus, pale mucus membranes, and markedly prolonged activated clotting time. Affected lambs had consistently prolonged 1-stage prothrombin times and activated partial thromboplastin times that supported a defect in the common pathway or defects in both the intrinsic and extrinsic pathway of the coagulation cascade. Decreased activity of vitamin K-dependent procoagulant factors II, VII, IX, and X in male and female lambs suggested either a defect of the hepatic enzyme gamma-glutamyl carboxylase, or vitamin K(1) 2,3 epoxide reductase. Affected lamb hepatic gamma-glutamyl carboxylase activity was markedly decreased compared with that of age- and sex-matched control lambs, while vitamin K(1) 2,3 epoxide reductase and glucose-6-phosphatase activities were similar between an affected and normal lamb. Subcutaneous vitamin K(1) supplementation did not increase vitamin K-dependent procoagulant factor activities in 3 lambs administered vitamin K(1) daily. These data confirm defective gamma-glutamyl carboxylase activity as the cause of impaired coagulation of sheep in this flock. This flock represents the only viable animal model of hereditarily defective gamma-glutamyl carboxylase activity. SN - 0300-9858 UR - https://www.unboundmedicine.com/medline/citation/16966451/Defective_gamma_glutamyl_carboxylase_activity_and_bleeding_in_Rambouillet_sheep_ L2 - http://journals.sagepub.com/doi/full/10.1354/vp.43-5-726?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -