Inadvertent Propagation of Factor VII Deficiency in a Canine Mucopolysaccharidosis Type I Research Breeding Colony

Carlstrom Lucas P; Jens Jackie K; Dobyns Marley E; Passage Merry; Dickson Patricia I; Ellinwood N Matthew

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Inadvertent Propagation of Factor VII Deficiency in a Canine Mucopolysaccharidosis Type I Research Breeding Colony

机译：在犬I型粘多糖贮积症研究繁殖群中无意传播因子VII缺乏症

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Issuesofcostandgeneticscanresultininbreedingofcaninegeneticdiseasecolonies.Beaglesoftenareusedtomaintainsuchcolonies,providingstockforoutcrosses.FactorVII(FVII)deficiencyisahemostaticdisorderfoundatincreasedfrequencyinbeaglesandhasbeencharacterizedattheDNAlevel.DeficiencyofFVIIpresentsobstaclesincoloniesfoundedwithbeagles.AninitialfindingofaFVII-deficientpupfromalongstandingcolonypromptedustoevaluateFVIIdeficiencyfullyinthiscolony.Currentandarchivalrecordsandtissueswereusedtoreconstructthecolonypedigree,assessthecontributionfrombeagles,andtestsamplestodocumentthesourceandfrequencyofthemutantFVIIallele.AspartofthisstudywedevelopedaPCR-baseddiagnosticassaythatwassimplerthanwhatwaspreviouslyavailable.Pedigreeanalysisrevealedafoundereffectimplicatingbeaglesthatledtohighfrequency(55%)ofthemutantallele.Inaddition,affectedanimalswereidentified.Thecompletepictureoftheclinicaleffectwithinthecolonyremainsunclear,butunusualneonatalpresentations,includinghemoabdomen,haveoccurredinpupsaffectedwithFVIIdeficiency.UseofaPCR-baseddiagnosticassaytoscreenallpotentialbeaglebreedingstockwillpreventsimilaroccurrencesofFVIIdeficiencyinfuturecanineresearchcolonies.

机译：Issuesofcostandgeneticscanresultininbreedingofcaninegeneticdiseasecolonies.Beaglesoftenareusedtomaintainsuchcolonies，providingstockforoutcrosses.FactorVII（FVII）deficiencyisahemostaticdisorderfoundatincreasedfrequencyinbeaglesandhasbeencharacterizedattheDNAlevel.DeficiencyofFVIIpresentsobstaclesincoloniesfoundedwithbeagles.AninitialfindingofaFVII-deficientpupfromalongstandingcolonypromptedustoevaluateFVIIdeficiencyfullyinthiscolony.Currentandarchivalrecordsandtissueswereusedtoreconstructthecolonypedigree，assessthecontributionfrombeagles，andtestsamplestodocumentthesourceandfrequencyofthemutantFVIIallele.AspartofthisstudywedevelopedaPCR-baseddiagnosticassaythatwassimplerthanwhatwaspreviouslyavailable.Pedigreeanalysisrevealedafoundereffectimplicatingbeaglesthatledtohighfrequency（55％）ofthemutantallele.Inaddition，affectedanimalswereidentified.Thecompletepictureoftheclinicaleffectwithinthecolonyremainsunclear，butunusualneonatalpresentations，includinghemoabdomen，haveoccurredinpupsaffectedwithF VII缺陷。使用基于PCR的诊断方法来筛选所有潜在的比格犬繁殖种，可以防止将来的犬科动物研究菌落出现FVII缺陷的类似情况。

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《Comparative Medicine》 |2009年第4期|共页
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Carlstrom Lucas P; Jens Jackie K; Dobyns Marley E; Passage Merry; Dickson Patricia I; Ellinwood N Matthew;
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中图分类畜牧、动物医学、狩猎、蚕、蜂;
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1. Inadvertent propagation of factor VII deficiency in a canine mucopolysaccharidosis type I research breeding colony [J] . Carlstrom LP, Jens JK, Dobyns ME, Comparative Medicine . 2009,第4期

机译：犬粘多糖贮积病I型研究繁殖群体中因子VII缺乏症的无意繁殖
2. Central Nervous System Delivery of Helper-Dependent Canine Adenovirus Corrects Neuropathology and Behavior in Mucopolysaccharidosis Type VII Mice [J] . Lorena Ariza, Lydia Gimenez-Llort, Aurelie Cubizolle, Human gene therapy. Clinical development . 2014,第3期

机译：依赖于依赖犬的腺病毒的中枢神经系统传递纠正了VII型黏多糖贮积病小鼠的神经病理学和行为。
3. Histopathologic changes of the ear in canine models of mucopolysaccharidosis types I and VII. [J] . Hordeaux J., Deniaud J., Bemelmans I., Veterinary Pathology . 2011,第3期

机译：在I型和VII型粘多糖贮积病犬模型中，耳朵的组织病理学变化。
4. Factor VII Deficiency:Clinical Manifestation and Molecular Genetics of 718 Subjects with FVII Gene Mutations [C] . F. H. Herrmann, K. Wulff, G. Auerswald, Hemophilia Symposium . 2008

机译：因子vii缺乏：718个受试者的临床表现与FVII基因突变的临床表现和分子遗传学
5. Characterization of human mesenchymal stem cells and therapeutic application to a xenotransplant model of mucopolysaccharidosis type VII. [D] . Meyerrose, Todd E. 2007

机译：人间充质干细胞的特征及其在VII型粘多糖贮积症异种移植模型中的治疗应用。
6. Inadvertent Propagation of Factor VII Deficiency in a Canine Mucopolysaccharidosis Type I Research Breeding Colony [O] . Lucas P Carlstrom, Jackie K Jens, Marley E Dobyns, 2009

机译：在犬I型粘多糖贮积病研究繁殖群中无意传播因子VII缺乏症
7. Central Nervous System Delivery of Helper-Dependent Canine Adenovirus Corrects Neuropathology and Behavior in Mucopolysaccharidosis Type VII Mice [O] . Lorena Ariza, Lydia Giménez-Llort, Aurélie Cubizolle, 2014

机译：辅助依赖的犬腺病毒的中枢神经系统递送校正粘附型VII小鼠的神经病理学和行为
8. Endotoxin-Induced Alterations in Canine Granulopoiesis: Colony-Stimulating Factor, Colony-Forming Cells in Culture, and Growth of Cells in Diffusion Chambers. [R] . MacVittie, T. J., Walker, R. I. 1977

机译：内毒素诱导犬粒细胞生成的改变：集落刺激因子，培养中的集落形成细胞和扩散室中细胞的生长。

Inadvertent Propagation of Factor VII Deficiency in a Canine Mucopolysaccharidosis Type I Research Breeding Colony

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