Collagen-VI supplementation by cell transplantation improves muscle regeneration in Ullrich congenital muscular dystrophy model mice.

ABSTRACT:

Background

Mesenchymal stromal cells (MSCs) function as supportive cells on skeletal muscle homeostasis through several secretory factors including type 6 collagen (COL6). Several mutations of COL6A1, 2, and 3 genes cause Ullrich congenital muscular dystrophy (UCMD). Skeletal muscle regeneration deficiency has been reported as a characteristic phenotype in muscle biopsy samples of human UCMD patients and UCMD model mice. However, little is known about the COL6-dependent mechanism for the occurrence and progression of the deficiency. The purpose of this study was to clarify the pathological mechanism of UCMD by supplementing COL6 through cell transplantation.

Methods

To test whether COL6 supplementation has a therapeutic effect for UCMD, in vivo and in vitro experiments were

SUBMITTER: Takenaka-Ninagawa N 

PROVIDER: S-EPMC8351132 | biostudies-literature | 2021 Aug

REPOSITORIES: biostudies-literature