Myotilin: a prominent marker of myofibrillar remodelling.
Myofibrillar remodelling with insertion of sarcomeres is a typical feature of biopsies taken from persons suffering of exercise-induced delayed onset muscle soreness. Here we studied the presence of the sarcomeric protein myotilin in eccentric exercise related lesions. Myotilin is a component of sarcomeric Z-discs and it binds several other Z-disc proteins, i.e. alpha-actinin, filamin C, F-actin and FATZ. Myotilin has previously been shown to be present in nemaline rods and central cores and to be mutated in limb girdle muscular dystrophy 1A (LGMD1A) and in a subset of myofibrillar myopathies, indicating an important role in Z-disc maintenance. Our findings on non-diseased muscle affected by eccentric exercise give new information on how myotilin is associated to myofibrillar components upon remodelling. We show that myotilin was present in increased amount in lesions related to Z-disc streaming and events leading to insertion of new sarcomeres in pre-existing myofibrils and can therefore be used as a marker for myofibrillar remodelling. Interestingly, myotilin is preferentially associated with F-actin rather than with the core Z-disc protein alpha-actinin during these events. This suggests that myotilin has a key role in the dynamic molecular events mediating myofibrillar assembly in normal and diseased skeletal muscle.
Carlsson
Lena
L
Department of Integrative Medical Biology, Section for Anatomy, Umea University, Sweden.
Yu
Ji-Guo
JG
Moza
Monica
M
Carpén
Olli
O
Thornell
Lars-Eric
LE
D000222:Adaptation, Physiological; D000328:Adult; D015415:Biomarkers / Q000378:metabolism; D064211:Connectin; D003598:Cytoskeletal Proteins / Q000378:metabolism*; D015444:Exercise; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D008297:Male; D008840:Microfilament Proteins; D009124:Muscle Proteins / Q000378:metabolism*; D009210:Myofibrils / Q000502:physiology* / Q000648:ultrastructure; D012518:Sarcomeres / Q000378:metabolism / Q000648:ultrastructure
D015415:Biomarkers; D064211:Connectin; D003598:Cytoskeletal Proteins; C577290:MYOT protein, human; D008840:Microfilament Proteins; D009124:Muscle Proteins