Myopalladin promotes muscle growth through modulation of the serum response factor pathway.

ABSTRACT:

Background

Myopalladin (MYPN) is a striated muscle-specific, immunoglobulin-containing protein located in the Z-line and I-band of the sarcomere as well as the nucleus. Heterozygous MYPN gene mutations are associated with hypertrophic, dilated, and restrictive cardiomyopathy, and homozygous loss-of-function truncating mutations have recently been identified in patients with cap myopathy, nemaline myopathy, and congenital myopathy with hanging big toe.

Methods

Constitutive MYPN knockout (MKO) mice were generated, and the role of MYPN in skeletal muscle was studied through molecular, cellular, biochemical, structural, biomechanical, and physiological studies in vivo and in vitro.

Results

MKO mice were 13% smaller compared with wild-type controls and exhibited a 48% redu

SUBMITTER: Filomena MC 

PROVIDER: S-EPMC7015241 | biostudies-literature | 2020 Feb

REPOSITORIES: biostudies-literature