Novel Pathologic Findings Associated with Urinary Retention in a Mouse Model of Mucopolysaccharidosis Type IIIB

Gografe Sylvia I; Sanberg Paul R; Chamizo Wilfredo; Monforte Hector; Garbuzova-Davis Svitlana

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Novel Pathologic Findings Associated with Urinary Retention in a Mouse Model of Mucopolysaccharidosis Type IIIB

机译：与IIIB型黏多糖贮积病小鼠模型中尿Re留相关的新病理发现。

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MucopolysaccharidosistypeIIIB(MPSIIIB;SanfilipposyndrometypeB)isametabolicdisorderwithdevastatingclinicalcharacteristicsstartinginearlychildhoodandleadingtoprematuredeath.Aknockoutmousestrainwasdevelopedthatmodelsthisdisease.MiceofthestrainB6.129S6-Naglusuptm1Efn/sup/Jareinvaluableforinvestigatingpathogenesisandpossibletreatmentmodalities.However,themousestrainalsoexhibitssomeobjectionablephenotypicfeatures.Onesuchfeature,urinaryretention,notonlyisatypicalofhumanMPSIIIBbutoftenleadstoearlyterminationofexperimentsduetoanimalwelfareconcerns.Theaimofthisstudywastoinvestigateabnormalitiesassociatedwiththeurinaryretention.Necropsieswereperformedon9-mo-oldmice;urinalysis,hematologyandbloodchemistryparameterswereevaluated,andurogenitalspecimensweremicroscopicallyexamined.Histopathologicexaminationsofurinarytractspecimensprovedilluminatingregardingpathologyintheurinarytract.Alargemononuclearcellinfiltratewasdiscoveredinmutantmiceofbothsexes,morepronouncedinfemalescomparedwithmalemice.Theinfiltratecomprisesoflargeroundedorpolygonalcellswithgenerousvariablyvacuolated,granulareosinophiliccytoplasmandsmallroundvesicularnuclei.Thesecellswerepresentthroughoutandexpandtheinterstitiumofthelowerurinarytract.Eitherthisresultsinextrinsiccompressionofthelumenoftheurethra,eventuallyleadingtoobstructiveuropathy,bladderhyperdistension,andurinaryretentionorpossiblyinterfereswiththeneurogeniccomponentofmicturitionneedstobefurtherinvestigated.ThenovelfindingofanunexpectedmononuclearcellinfiltrateintheurinarytractintheknockoutmiceB6.129S6-Naglusuptm1Efn/sup/Jisreported.

机译：MucopolysaccharidosistypeIIIB（MPSIIIB; SanfilipposyndrometypeB）isametabolicdisorderwithdevastatingclinicalcharacteristicsstartinginearlychildhoodandleadingtoprematuredeath.Aknockoutmousestrainwasdevelopedthatmodelsthisdisease.MiceofthestrainB6.129S6-的Naglu ^{tm1Efn /Jareinvaluableforinvestigatingpathogenesisandpossibletreatmentmodalities.However,themousestrainalsoexhibitssomeobjectionablephenotypicfeatures.Onesuchfeature,urinaryretention,notonlyisatypicalofhumanMPSIIIBbutoftenleadstoearlyterminationofexperimentsduetoanimalwelfareconcerns.Theaimofthisstudywastoinvestigateabnormalitiesassociatedwiththeurinaryretention.Necropsieswereperformedon9-mo-oldmice;urinalysis,hematologyandbloodchemistryparameterswereevaluated,andurogenitalspecimensweremicroscopicallyexamined。经尿道病理学检查证实了泌尿道病理学检查。在两个性腺的不育小鼠中均发现了较大的单核细胞浸润液，与公鼠相比，女性的发音更明显。 filtratecomprisesoflargeroundedorpolygonalcellswithgenerousvariablyvacuolated，granulareosinophiliccytoplasmandsmallroundvesicularnuclei.Thesecellswerepresentthroughoutandexpandtheinterstitiumofthelowerurinarytract.Eitherthisresultsinextrinsiccompressionofthelumenoftheurethra，eventuallyleadingtoobstructiveuropathy，bladderhyperdistension，andurinaryretentionorpossiblyinterfereswiththeneurogeniccomponentofmicturitionneedstobefurtherinvestigated.ThenovelfindingofanunexpectedmononuclearcellinfiltrateintheurinarytractintheknockoutmiceB6.129S6-的Naglu ^{tm1Efn / Jisreported。}}

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《Comparative Medicine》 |2009年第2期|共页
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Gografe Sylvia I; Sanberg Paul R; Chamizo Wilfredo; Monforte Hector; Garbuzova-Davis Svitlana;
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中图分类畜牧、动物医学、狩猎、蚕、蜂;
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1. Novel pathologic findings associated with urinary retention in a mouse model of mucopolysaccharidosis type IIIB [J] . Gografe SI, Sanberg PR, Chamizo W, Comparative Medicine . 2009,第2期

机译：与IIIB型粘多糖贮积症小鼠模型中尿retention留有关的新病理学发现
2. Time- and dose -dependentnormalization of pathological lysosomal storage and biochemistry in the mucopolysaccharidosis IIIB (MPS IIIB, Sanfilippo syndrome type B) mouse model by intracerebroventricular enzyme replacement therapy with BMN 250, a NAGLU-IGF2 fusion pro [J] . Aoyagi-Scharber Mika, Vincelette Jon, Lawrence Roger, Molecular genetics and metabolism . 2016,第2期

机译：NAGLU-IGF2融合蛋白BMN 250的脑室内脑酶替代疗法在粘多糖贮积症IIIB（MPS IIIB，Sanfilippo综合征B型）小鼠模型中的病理溶酶体贮藏和生物化学的时间和剂量依赖性归一化
3. Histologic characterization of the progression of central nervous system pathology in the mucopolysaccharidosis IIIB (MPS IIIB, Sanfilippo syndrome type B) mouse model and bio-distribution and efficacy of the intracerebroventricular enzyme replacement therapy, BMN 250 [J] . Bagri Anil, Harmon Danielle, Vincelette Jon, Molecular genetics and metabolism . 2016,第2期

机译：黏多糖贮积症IIIB（MPS IIIB，Sanfilippo综合征B型）小鼠模型的中枢神经系统病理学进展的组织学特征以及脑室内脑酶替代疗法（BMN 250）的生物分布和功效
4. Detection of ganglioside lipid species in the brain of mucopolysaccharidosis type II mouse by imaging mass spectrometry [C] . Martin Dufresne, Daniel Guneysu, Nathan H. Patterson, ASMS Conference on Mass Spectrometry and Allied Topics . 2016

机译：通过成像质谱法检测粘性多种子型小鼠脑脑中的神经胶状物脂质物种
5. Mucopolysaccharidosis type IIIB and GalNAc Transferase double knockout mice and additional studies in gene therapy and animal models to assess pathogenesis and therapy. [D] . Mohammed, Eman. 2012

机译：IIIB型粘多糖贮积病和GalNAc转移酶双敲除小鼠以及基因治疗和动物模型的其他研究，以评估发病机理和治疗方法。
6. Neuropathology in Mouse Models of Mucopolysaccharidosis Type I, IIIA and IIIB [O] . Fiona L. Wilkinson, Rebecca J. Holley, Kia J. Langford-Smith, 2009

机译：I，IIIA和IIIB型粘多糖贮积症小鼠模型的神经病理学
7. Neuropathology in Mouse Models of Mucopolysaccharidosis Type I, IIIA and IIIB [O] . Wilkinson, Fiona L., Holley, Rebecca J., Langford-Smith, Kia J., 2012

机译：I，IIIA和IIIB型粘多糖贮积症小鼠模型的神经病理学

Novel Pathologic Findings Associated with Urinary Retention in a Mouse Model of Mucopolysaccharidosis Type IIIB

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