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患有囊性纤维化的幼兔的内分泌病理学

Endocrine pathology in young rabbits with cystic fibrosis.

作者信息

Liang Xiubin, Hou Xia, Chen Y Eugene, Jin Jian-Ping, Zhang Kezhong, Xu Jie

机构信息

Center for Advanced Models for Translational Sciences and Therapeutics, University of Michigan Medical Center, University of Michigan Medical School, Ann Arbor, MI, 48109, USA.

Department of Physiology, Wayne State University School of Medicine, Detroit, MI, 48201, USA.

出版信息

eGastroenterology. 2024 Nov;2(4). doi: 10.1136/egastro-2024-100102. Epub 2024 Nov 10.

DOI:10.1136/egastro-2024-100102
PMID:39605883
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11594368/
Abstract

BACKGROUND

Cystic fibrosis (CF) is an autosomal recessive genetic disorder caused by loss-of-function mutations in the transmembrane conductance regulator gene. CF-related pancreatic lesions are known to cause exocrine dysfunctions such as pancreatic insufficiency, and endocrine dysfunctions, including CF related diabetes. In a previous study, we generated CF rabbits using CRISPR/Cas9-mediated gene editing.

METHODS

CF rabbits were subjected to histological analysis with a focus on CF associated pancreatic lesions. Endocrine function related assays were conducted to evaluate CF related pancreatic endocrine disorders in these animals.

RESULTS

We report that CF rabbits develop spontaneous pancreatic lesions at a young age, characterised by pancreatic inflammation and fibrosis, vacuolar degeneration, epithelium mucus-secretory cell metaplasia, and pancreatic duct dilation. The size of the pancreatic islets in the CF rabbits is significantly smaller than that of the wild type animals. Consistent with these pathological findings, young CF rabbits exhibited signs of pancreatic endocrine related disorders such as lower insulin levels and impaired glucose metabolism.

CONCLUSIONS

Our results suggest that the CF rabbit could serve as a valuable model for translational research on CF related pancreatic endocrine dysfunction.

摘要

背景

囊性纤维化（CF）是一种常染色体隐性遗传疾病，由跨膜电导调节基因的功能丧失突变引起。已知与CF相关的胰腺病变会导致外分泌功能障碍，如胰腺功能不全，以及内分泌功能障碍，包括CF相关糖尿病。在先前的一项研究中，我们使用CRISPR/Cas9介导的基因编辑技术培育出了CF兔。

方法

对CF兔进行组织学分析，重点关注与CF相关的胰腺病变。进行内分泌功能相关检测，以评估这些动物中与CF相关的胰腺内分泌紊乱情况。

结果

我们报告称，CF兔在年轻时会自发出现胰腺病变，其特征为胰腺炎症和纤维化、空泡变性、上皮黏液分泌细胞化生以及胰管扩张。CF兔的胰岛大小明显小于野生型动物。与这些病理结果一致，年轻的CF兔表现出胰腺内分泌相关紊乱的迹象，如胰岛素水平降低和糖代谢受损。

结论

我们的结果表明，CF兔可作为CF相关胰腺内分泌功能障碍转化研究的有价值模型。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9432/11729302/42ba0d7828a7/egastro-2-4-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9432/11729302/93d43cbc6040/egastro-2-4-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9432/11729302/4aa568b65e40/egastro-2-4-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9432/11729302/42ba0d7828a7/egastro-2-4-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9432/11729302/93d43cbc6040/egastro-2-4-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9432/11729302/4aa568b65e40/egastro-2-4-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9432/11729302/42ba0d7828a7/egastro-2-4-g003.jpg

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JCI Insight. 2024 Feb 15;9(6):e165826. doi: 10.1172/jci.insight.165826.
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Cystic Fibrosis.囊性纤维化
N Engl J Med. 2023 Nov 2;389(18):1693-1707. doi: 10.1056/NEJMra2216474.
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Elexacaftor-Tezacaftor-Ivacaftor: A Life-Changing Triple Combination of CFTR Modulator Drugs for Cystic Fibrosis.依列卡福妥-替扎卡福妥-依伐卡托：用于囊性纤维化的改变人生的三联组合CFTR调节剂药物。
Pharmaceuticals (Basel). 2023 Mar 8;16(3):410. doi: 10.3390/ph16030410.
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Cystic fibrosis rabbits develop spontaneous hepatobiliary lesions and CF-associated liver disease (CFLD)-like phenotypes.囊性纤维化兔会出现自发性肝胆病变以及类似囊性纤维化相关肝病（CFLD）的表型。
PNAS Nexus. 2022 Dec 23;2(1):pgac306. doi: 10.1093/pnasnexus/pgac306. eCollection 2023 Jan.
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Early developmental phenotypes in the cystic fibrosis sheep model.囊性纤维化绵羊模型中的早期发育表型。
FASEB Bioadv. 2022 Oct 31;5(1):13-26. doi: 10.1096/fba.2022-00085. eCollection 2023 Jan.
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