Next Article in Journal
The Vicious Cross-Talk between Tumor Cells with an EMT Phenotype and Cells of the Immune System
Previous Article in Journal
A Critical E-box in Barhl1 3′ Enhancer Is Essential for Auditory Hair Cell Differentiation
Previous Article in Special Issue
Dermatopontin in Skeletal Muscle Extracellular Matrix Regulates Myogenesis
Article Menu
Issue 5 (May) cover image

Export Article

Open AccessArticle

Overexpression of miR-29 Leads to Myopathy that Resemble Pathology of Ullrich Congenital Muscular Dystrophy

Beijing Advanced Innovation Center for Food Nutrition and Human Health, College of Biological Science, China Agricultural University, Beijing 100193, China
The State Key Laboratory for Agrobiotechnology, College of Biological Sciences, China Agricultural University, Beijing 100193, China
The Institute of Bioengineering and Technology, Inner Mongolia University of Science and Technology, Baotou 014010, China
Author to whom correspondence should be addressed.
Cells 2019, 8(5), 459;
Received: 6 April 2019 / Revised: 11 May 2019 / Accepted: 13 May 2019 / Published: 15 May 2019
PDF [3354 KB, uploaded 23 May 2019]


Ullrich congenital muscular dystrophy (UCMD) bring heavy burden to patients’ families and society. Because the incidence of this disease is very low, studies in patients are extremely limited. Animal models of this disease are indispensable. UCMD belongs to extracellular matrix-related diseases. However, the disease models constructed by knocking out some pathogenic genes of human, such as the Col6a1, Col6a2, or Col6a3 gene, of mice could not mimic UCMD. The purpose of this study is to construct a mouse model which can resemble the pathology of UCMD. miR-29 is closely related to extracellular matrix deposition of tissues and organs. To address this issue, we developed a mouse model for overexpression miR-29 using Tet-on system. In the muscle-specific miR-29ab1 cluster transgenic mice model, we found that mice exhibited dyskinesia, dyspnea, and spinal anomaly. The skeletal muscle was damaged and regenerated. At the same time, we clarify the molecular mechanism of the role of miR-29 in this process. Different from human, Col4a1 and Col4a2, target genes of miR-29, are the key pathogenic genes associating with these phenotypes. This mouse model simulates the human clinical and pathological characteristics of UCMD patients and is helpful for the subsequent research and treatment of UCMD. View Full-Text
Keywords: UCMD; disease model; miR-29; dysplasia; collagen UCMD; disease model; miR-29; dysplasia; collagen

Figure 1

This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).

Supplementary material


Share & Cite This Article

MDPI and ACS Style

Liu, C.; Li, L.; Ge, M.; Gu, L.; Wang, M.; Zhang, K.; Su, Y.; Zhang, Y.; Liu, C.; Lan, M.; Yu, Y.; Wang, T.; Li, Q.; Zhao, Y.; Yu, Z.; Li, N.; Meng, Q. Overexpression of miR-29 Leads to Myopathy that Resemble Pathology of Ullrich Congenital Muscular Dystrophy. Cells 2019, 8, 459.

Show more citation formats Show less citations formats

Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Related Articles

Article Metrics

Article Access Statistics



[Return to top]
Cells EISSN 2073-4409 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
Back to Top