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Characterisation of Lamp2-deficient rats for potential new animal model of Danon disease.

Authors: Ma, Shuoyi  Zhang, Miao  Zhang, Shuai  Wang, Jing  Zhou, Xia  Guo, Guanya  Wang, Lu  Wang, Min  Peng, Zhengwu  Guo, Changcun  Zheng, Xiaohong  Zhou, Xinmin  Wang, Jingbo  Han, Ying 
Citation: Ma S, etal., Sci Rep. 2018 May 2;8(1):6932. doi: 10.1038/s41598-018-24351-w.
Pubmed: (View Article at PubMed) PMID:29720683
DOI: Full-text: DOI:10.1038/s41598-018-24351-w

Danon disease (DD) is caused by the absence or malfunction of lysosomal-associated membrane protein 2 (LAMP2). Although Lamp2-deficient mice and DD patients have similar characteristics, these mice have clear limitations and are clinically inconsistent. The aim of our paper is to outline the characteristics of Lamp2-deficient rats and to contrast this model with currently available DD mouse models. The baseline levels of some serum enzymes were elevated in Lamp2y/- rats along with hypercholesterolemia and hyperglycaemia at 8 weeks. Echocardiography showed that IVSd (1.500¿±¿0.071 vs. 2.200¿±¿1.147, P¿<¿0.01) and LVPWd (1.575¿±¿0.063 vs. 1.850¿±¿0.029, P¿<¿0.01) were significantly increased, and GCS (-13.20¿±¿0.4814 vs. -6.954¿±¿0.665) and GRS (21.42¿±¿1.807 vs. 7.788¿±¿1.140) were sharply decreased. Meanwhile, substantial myocyte disruption, hypertrophic muscle fibres, interstitial fibrosis and microvascular hyperplasia could be observed in the heart tissue. Lamp2y/- rats also displayed abnormal behaviours in the open field and fear conditioning tests. Notably, Lamp2y/- rats manifested other system dysfunctions, such as retinopathy, chronic kidney injury and sterility. Based on these results, Lamp2-deficient rats exhibited greater similarity to DD patients in terms of onset and multisystem lesions than did mouse models, and these rats could be used as a valuable animal model for DD.

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CRRD Object Information
CRRD ID: 13703117
Created: 2018-08-02
Species: All species
Last Modified: 2018-08-02
Status: ACTIVE



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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.