AbstractTwo cystic fibrosis (CF) rat models, one carrying the common Phe508del mutation and the other a nonsense cystic fibrosis transmembrane conductance regulator (CFTR) mutation (knockout) were previously characterised. Although relevant CFTR mRNA reductions were present in the lung, no overt CF lung disease was observed.

摘要先前已经表征了两种囊性纤维化（CF）大鼠模型，一种携带常见的Phe508del突变，另一种携带无意义的囊性纤维化跨膜电导调节剂（CFTR）突变（敲除）。尽管肺中存在相关的CFTR mRNA减少，但未观察到明显的CF肺病。

This study used flexiVent lung mechanic assessment and regional ventilation assessment via X-ray velocimetry (XV) functional imaging to assess the lung phenotype in both models. To determine the sensitivity of XV regional ventilation imaging, the effect of a localised physical obstruction (delivery of agar beads to part of the lungs) on lung ventilation was examined.

本研究通过X射线测速仪（XV）功能成像使用flexiVent肺力学评估和区域通气评估来评估两种模型中的肺表型。为了确定XV区域通气成像的敏感性，检查了局部物理阻塞（将琼脂珠递送至部分肺部）对肺通气的影响。

At baseline, Phe508del and knockout CF rats had a lower inspiratory capacity, total respiratory system compliance, and static compliance than wildtype rats. Following agar bead delivery all XV ventilation parameters were altered, with substantial increases in poorly ventilated regions and ventilation heterogeneity.

在基线时，Phe508del和基因敲除CF大鼠的吸气能力，总呼吸系统顺应性和静态顺应性均低于野生型大鼠。琼脂珠递送后，所有XV通气参数均发生改变，通风不良区域和通气不均匀性显着增加。

XV ventilation maps accurately identified locations of bead-induced airflow changes. Despite unremarkable lung histopathology, this study indicated that CF rats display altered respiratory mechanics, with CF rats needing to exert additional effort to expand and deflate their lungs due to increased stiffness.

XV通风图准确识别了珠子引起的气流变化的位置。尽管肺组织病理学不明显，但这项研究表明CF大鼠表现出呼吸力学的改变，CF大鼠由于僵硬程度增加，需要付出额外的努力来扩大和缩小肺部。

This study demonstrated the utility of XV imaging providing spatial lung ventilation information..

这项研究证明了XV成像提供空间肺通气信息的实用性。。

IntroductionCystic fibrosis (CF) is a life-shortening genetic disease that affects multiple organs1, but lung disease causes the majority of morbidity and mortality. The CF transmembrane conductance regulator (CFTR) is a chloride and bicarbonate ion transport channel on epithelial surfaces that contributes to the absorption and secretion of ions.

引言囊性纤维化（CF）是一种缩短寿命的遗传性疾病，影响多个器官1，但肺部疾病导致大部分发病率和死亡率。。

Pathogenic variants of the CFTR gene result in defective CFTR function and altered chloride ion movement, leading to dehydration of the airway surface liquid and impaired mucociliary clearance2. Bacteria entering the lungs adhere to the accumulated mucus and are unable to be cleared, resulting in a persistent cycle of infection and inflammation.

CFTR基因的致病变异导致CFTR功能缺陷和氯离子运动改变，导致气道表面液体脱水和粘膜纤毛清除受损2。进入肺部的细菌粘附在累积的粘液上，无法清除，导致持续的感染和炎症循环。

The combination of mucus stasis, bacterial infections and inflammation contribute to altered lung architecture and airway remodelling, resulting in an obstructive lung disease and progressive decline in lung function. Lung function tests such as spirometry, body plethysmography and multiple breath washout in those with CF are important tools for monitoring pulmonary exacerbation, disease progression and treatment outcomes3.

粘液淤滞，细菌感染和炎症的结合导致肺结构改变和气道重塑，导致阻塞性肺病和肺功能逐渐下降。CF患者的肺功能检查，如肺活量测定，体积描记术和多次呼吸冲洗，是监测肺部恶化，疾病进展和治疗结果的重要工具3。

In humans these are often combined with lung imaging via computed tomography (CT), or more recently magnetic resonance imaging, to identify structural lung disease including bronchiectasis, mucus plugging, atelectasis, bronchial wall thickening, air trapping and ventilation inhomogeneity4.Numerous animal models of CF have been developed, with each displaying a varied CF phenotype5,6.

在人类中，这些通常通过计算机断层扫描（CT）或最近的磁共振成像与肺部成像相结合，以识别结构性肺部疾病，包括支气管扩张，粘液堵塞，肺不张，支气管壁增厚，空气潴留和通气不均4。已经开发了许多CF动物模型，每个模型都显示出不同的CF表型5,6。

Recently, two CF rat models, one carrying the common Phe508del mutation and the other a CFTR knockout were generated in Adelaide, South Australia7. Both models exhibit reduced survival, intestinal obstructions, defects in the male reproductive tract, and a CF bioelectrical profile in the.

最近，在南澳大利亚阿德莱德产生了两种CF大鼠模型，一种携带常见的Phe508del突变，另一种携带CFTR基因敲除7。两种模型均表现出存活率降低，肠梗阻，男性生殖道缺陷以及CF生物电特征。

Data availability

数据可用性

The data can be found at https://doi.org/10.25909/25734549.

https://doi.org/10.25909/25734549.

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Download referencesAcknowledgementsThe authors acknowledge the facilities and scientific and technical assistance of the National Imaging Facility, a National Collaborative Research Infrastructure Strategy (NCRIS) capability, at the Large Animal Research and Imaging Facility, South Australian Health and Medical Research Institute.

下载参考文献致谢作者感谢国家成像设施（National Imaging Facility）的设施和科学技术援助，这是一种国家合作研究基础设施战略（NCRIS）能力，位于南澳大利亚健康与医学研究所的大型动物研究和成像设施。

The authors acknowledge Wick Lakshantha and Ryan O’Hare Doig for operating the Permetium small animal scanner. This project built on experiments M11727, M12061 and M12926 performed on the Imaging and Medical Beamline at the Australian Synchrotron, and the authors thank Kaye Morgan, Marcus Kitchen, and Bernadette Boog for their input.FundingStudies were supported by NHMRC Project Grant GNT1160011, Cystic Fibrosis Foundation Grant DONNEL21GO, and the Medical Research Future Fund Grant RFRHPSI000013.Author informationAuthors and AffiliationsRobinson Research Institute, University of Adelaide, Adelaide, SA, AustraliaNicole Reyne, Ronan Smith, Patricia Cmielewski, David Parsons & Martin DonnelleyAdelaide Medical School, University of Adelaide, Adelaide, SA, AustraliaNicole Reyne, Ronan Smith, Patricia Cmielewski, David Parsons & Martin DonnelleyRespiratory and Sleep Medicine, Women’s and Children’s Hospital, Adelaide, SA, AustraliaNicole Reyne, Ronan Smith, Patricia Cmielewski, David Parsons & Martin Donnelley4DMedical, Melbourne, VIC, AustraliaNina Eikelis & Piraveen PirakalathananSCIREQ Scientific Respiratory Equipment Inc, Montreal, QC, CanadaMark LawrenceBiostatistics Unit, South Australian Health and Medical Research Institute, Adelaide, SA, AustraliaJennie LouiseAuthorsNicole ReyneView author publicationsYou can also search for this author in.

作者感谢Wick Lakshantha和Ryan O'Hare Doig操作Permetium小动物扫描仪。该项目基于在澳大利亚同步加速器的成像和医疗光束线上进行的M11727、M12061和M12926实验，作者感谢Kaye Morgan、Marcus Kitchen和Bernadette Boog的投入。资助研究得到了NHMRC项目资助GNT1160011，囊性纤维化基金会资助DONNEL21GO和医学研究未来基金资助RFRHPSI000013的支持。作者信息作者和附属机构阿德莱德大学罗宾逊研究所，阿德莱德，南澳州，澳大利亚·雷恩，罗南·史密斯，帕特里夏·Cmielewski，大卫·帕森斯和马丁·唐纳利·阿德莱德医学院，阿德莱德大学，南澳大利亚·雷恩，罗南·史密斯，帕特里夏·Cmielewski，大卫·帕森斯和马丁·唐纳利呼吸和睡眠医学，妇女和儿童医院，阿德莱德，SA，澳大利亚雷恩，罗南·史密斯，帕特里夏·克米列夫斯基，大卫·帕森斯和马丁·唐纳利4DMedical，墨尔本，维多利亚州，澳大利亚埃克利斯和皮拉文·皮拉卡拉塔纳·西雷克科学呼吸设备公司，蒙特利尔，QC，加拿大马克·劳伦斯生物统计学部门，南澳大利亚健康与医学研究所，阿德莱德，SA，澳大利亚珍妮·路易丝·奥瑟斯·妮可·雷内维（NicoleReyneview）作者出版物您也可以在中搜索这位作者。

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PubMed Google ScholarContributionsN.R.: Conceptualisation, Data Curation, Formal analysis, Investigation, Methodology, Visualization, Writing—Original Draft. R.S.: Data Curation, Formal analysis, Investigation, Software, Visualization, Writing—Original Draft. P.C.: Investigation, Methodology, Data curation, Writing—Review and Editing.

PubMed谷歌学术贡献。R、 ：概念化，数据管理，形式分析，调查，方法论，可视化，撰写原稿。R、 数据管理，形式分析，调查，软件，可视化，撰写原稿。P、 C.：调查，方法论，数据管理，写作评论和编辑。

J.L., M.L., N.E., P.P.: Formal analysis, Writing – Review & Editing. D.P.: Conceptualisation, Formal analysis, Resources, Methodology, Supervision, Writing—Original Draft, Writing—Review and Editing. M.D.: Conceptualisation, Data Curation, Formal analysis, Funding acquisition, Investigation, Methodology, Supervision, Visualization, Writing—Original Draft.Corresponding authorCorrespondence to.

J、 L.，M.L.，N.E.，P.P.：正式分析，写作-评论和编辑。D、 P：概念化，形式分析，资源，方法论，监督，撰写原稿，撰写评论和编辑。M、 D.：概念化，数据管理，正式分析，资金获取，调查，方法论，监督，可视化，撰写原稿。对应作者对应。

Nicole Reyne.Ethics declarations

妮可·雷恩。道德宣言

Competing interests

相互竞争的利益

MD and DP were involved in the research development and validation of the XV technology and have personally purchased shares in 4DMedical. NE and PP are employed by 4DMedical. ML is employed by SCIREQ Scientific Respiratory Equipment Inc.

MD和DP参与了XV技术的研究开发和验证，并亲自购买了4DMedical的股份。。ML由SCIREQ Scientific Respiratory Equipment Inc.雇用。

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Reprints and permissionsAbout this articleCite this articleReyne, N., Smith, R., Cmielewski, P. et al. Assessment of respiratory mechanics and X-ray velocimetry functional imaging in two cystic fibrosis rat models.

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Sci Rep 14, 21646 (2024). https://doi.org/10.1038/s41598-024-71632-8Download citationReceived: 06 June 2024Accepted: 28 August 2024Published: 17 September 2024DOI: https://doi.org/10.1038/s41598-024-71632-8Share this articleAnyone you share the following link with will be able to read this content:Get shareable linkSorry, a shareable link is not currently available for this article.Copy to clipboard.

科学报告1421646（2024）。https://doi.org/10.1038/s41598-024-71632-8Download引文收到日期：2024年6月6日接受日期：2024年8月28日发布日期：2024年9月17日OI：https://doi.org/10.1038/s41598-024-71632-8Share本文与您共享以下链接的任何人都可以阅读此内容：获取可共享链接对不起，本文目前没有可共享的链接。复制到剪贴板。

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KeywordsCystic fibrosisAnimal modelsLungLung functionX-ray velocimetryflexiVent

关键词囊性纤维化动物模型肺功能X射线测速仪柔性

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