Role of mitochondria in the myopathy of juvenile dermatomyositis and implications for skeletal muscle calcinosis.

ABSTRACT:

Objectives

To elucidate mechanisms contributing to skeletal muscle calcinosis in patients with juvenile dermatomyositis.

Methods

A well-characterized cohorts of JDM (n = 68), disease controls (polymyositis, n = 7; juvenile SLE, n = 10, and RNP + overlap syndrome, n = 12), and age-matched health controls (n = 17) were analyzed for circulating levels of mitochondrial (mt) markers including mtDNA, mt-nd6, and anti-mitochondrial antibodies (AMAs) using standard qPCR, ELISA, and novel-in-house assays, respectively. Mitochondrial calcification of affected tissue biopsies was confirmed using electron microscopy and energy dispersive X-ray analysis. A human skeletal muscle cell line, RH30, was used to generate an in vitro calcification model. Intracellular calcification is measured

SUBMITTER: Duvvuri B 

PROVIDER: S-EPMC10330803 | biostudies-literature | 2023 Jul

REPOSITORIES: biostudies-literature