The neonatal sarcoplasmic reticulum Ca-ATPase gives a clue to development and pathology in human muscles

The sarcoplasmic/endoplasmic reticulum calcium ATPase 1 (SERCA1) has two muscle specific splice isoforms; SERCA1a in fast-type adult and SERCA1b in neonatal and regenerating skeletal muscles. At the protein level the only difference between these two isoforms is that SERCA1a has C-terminal glycine...

Teljes leírás

Elmentve itt :
Bibliográfiai részletek
Szerzők: Kósa Magdolna
Brinyiczki Kitti
van Damme Philip
Goemans Nathalie
Hancsak Károly
Mendler Luca
Zádor Ernő
Dokumentumtípus: Cikk
Megjelent: Chapman and Hall 2015
Sorozat:JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY 36 No. 2
doi:10.1007/s10974-014-9403-z

mtmt:2790554
Online Access:http://publicatio.bibl.u-szeged.hu/10871
Leíró adatok
Tartalmi kivonat:The sarcoplasmic/endoplasmic reticulum calcium ATPase 1 (SERCA1) has two muscle specific splice isoforms; SERCA1a in fast-type adult and SERCA1b in neonatal and regenerating skeletal muscles. At the protein level the only difference between these two isoforms is that SERCA1a has C-terminal glycine while SERCA1b has an octapeptide tail instead. This makes the generation of a SERCA1a specific antibody not feasible. The switch between the two isoforms is a hallmark of differentiation so we describe here a method based on the signal ratios of the SERCA1b specific and pan SERCA1 antibodies to estimate the SERCA1b/SERCA1a dominance on immunoblot of human muscles. Using this method we showed that unlike in mouse and rat, SERCA1b was only expressed in pre-matured infant leg and arm muscles; it was replaced by SERCA1a in more matured neonatal muscles and was completely absent in human foetal and neonatal diaphragms. Interestingly, only SERCA1a and no SERCA1b were detected in muscles of 7-12 years old boys with Duchenne, a degenerative-regenerative muscular dystrophy. However, in adult patients with myotonic dystrophy type 2 (DM2), the SERCA1b dominated over SERCA1a. Thus the human SERCA1b has a different expression pattern from that of rodents and it is associated with DM2.
Terjedelem/Fizikai jellemzők:195-203
ISSN:0142-4319