Erratum: Treatment of Dystrophic mdx Mice with an ADAMTS-5 Specific Monoclonal Antibody Increases the Ex Vivo Strength of Isolated Fast Twitch Hindlimb Muscles. Biomolecules 2020, 10, 416

Abstract

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Single-cell transcriptomic analysis of the identity and function of fibro/adipogenic progenitors in healthy and dystrophic muscle

“… 35 Further, inhibition of ADAMTS5 activity in the DMD mouse model improves muscle function. 34 These demonstrate disease relevance of the FAP-enriched genes identified by our analysis.…”

“… 22 In the mouse model of DMD ( mdx ), Il33 and Adamts5 gene expression is increased while their inhibition can ameliorate the disease phenotype in the mice. 34 , 35 Using a combination of qRT-PCR, immunostaining, and RNA fluorescence in situ hybridization (FISH) analysis, we examined if disease severity in the dysferlin-deficient mouse corelates with the levels of FAP gene expression. In agreement with our previous report of disease stage-specific increase in FAP abundance in dysferlinopathic muscle, 26 qRT-PCR analysis revealed that, compared to WT (C57BL/6) mice, BLA/J mice at an early symptomatic stage (3-month-old) show increased FAP marker genes namely Pdgfra , Ly6a (SCA-1), C3 , Dpep1 , and Il33 , and this increased further in the advanced disease stage (12-month-old) ( Figure 5 A).…”

“… 39 We find expression of several FAP-enriched proteolysis regulatory proteins, including ADAMTS5, C3, and DPEP1 are increased in two different muscular dystrophy mouse models - dysferlinopathy (BLA/J) and DMD ( D2-mdx ). ADAMTS5 is a secreted chondroitin sulfate proteoglycanase that is known to be elevated in mdx muscle, 34 as well as in the serum of human DMD patient serum and in the serum of exercised mdx mice. 40 , 41 Secreted ADAMTS5 cleaves proteoglycans such as versican, which in turn can regulate myogenesis in vitro and in vivo.…”