The neonatal sarcoplasmic reticulum Ca2+-ATPase gives a clue to development and pathology in human muscles
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  • 作者:Magdolna Kósa ; Kitti Brinyiczki…
  • 关键词:SERCA1b ; SERCA1a ; New ; born muscle ; Myotonic dystrophy ; Duchenne muscular dystrophy ; Diaphragm
  • 刊名:Journal of Muscle Research and Cell Motility
  • 出版年:2015
  • 出版时间:April 2015
  • 年:2015
  • 卷:36
  • 期:2
  • 页码:195-203
  • 全文大小:1,093 KB
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    Sch?neich C, Viner RI, Ferrington DA, Bigelow DJ
  • 作者单位:Magdolna Kósa (1)
    Kitti Brinyiczki (2)
    Philip van Damme (3) (4) (5)
    Nathalie Goemans (6)
    Károly Hancsák (7)
    Luca Mendler (1)
    Ern? Zádor (1)

    1. Department of Biochemistry, Faculty of General Medicine, University of Szeged, Dóm tér 9, H-6720, Szeged, Hungary
    2. Department of Pathology, Faculty of General Medicine, University of Szeged, Szeged, Hungary
    3. Neurology Department, University Hospital Leuven, Louvain, Belgium
    4. Leuven Institute for Neurodegenerative Disorders (LIND), KU Leuven, Louvain, Belgium
    5. Vesalius Research Center VIB, Louvain, Belgium
    6. Department of Development and Regeneration, University Hospital Leuven, Herestraate 49, 3000, Louvain, Belgium
    7. Radnóti Miklós Experimental Grammar School, Szeged, Hungary
  • 刊物主题:Cell Biology; Proteomics; Animal Anatomy / Morphology / Histology; Biomedicine general;
  • 出版者:Springer Netherlands
  • ISSN:1573-2657
文摘
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 7-2?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.
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