Immunohistochemical analysis of human skeletal muscle AMP deaminase deficiency. Evidence of a correlation between the muscle HPRG content and the level of the residual AMP deaminase activity

Antonietta R M Sabbatini, Antonio Toscano, Mohammed Aguennouz, Daniela Martini, Enza Polizzi, Maria Ranieri-Raggi, Arthur J G Moir, Alba Migliorato, Olimpia Musumeci, Giuseppe Vita, Antonio Raggi

Research output: Contribution to journalArticle

Abstract

We have previously described that, in healthy human skeletal muscle, an anti-histidine-proline-rich-glycoprotein (HPRG) antibody selectively binds to type IIB fibers that are well known to contain the highest level of AMP deaminase (AMPD) activity, suggesting an association of the HPRG-like protein to the enzyme isoform M. The present paper reports an immunohistochemical study performed on human skeletal muscle biopsies from patients with AMPD deficiency and carried out utilizing both the anti-HPRG antibody and an anti-AMPD antibody specific for the isoform M. A correlation between the muscle content of the HPRG-like protein and the level of AMPD activity was demonstrated. In the specimens from patients with Acquired AMPD deficiency the HPRG-immunoreactivity was less intense than that shown by the control subjects and was related to the residual AMPD activity. The patients affected by Primary and Coincidental AMPD deficiency, which were characterized by an absence of enzyme activity and AMPD immunoreactivity, showed the lowest HPRG immunoreactivity that was clearly detectable by Western blot analysis, but not by immunohistochemistry. The interpretation of the significance of these observations suggests a physiological mutual dependence between skeletal muscle HPRG and AMPD polypeptides with regard to their stability.

Original languageEnglish
Pages (from-to)83-92
Number of pages10
JournalJournal of Muscle Research and Cell Motility
Volume27
Issue number1
DOIs
Publication statusPublished - Feb 2006

ASJC Scopus subject areas

  • Physiology
  • Clinical Biochemistry
  • Endocrinology
  • Cell Biology

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    Sabbatini, A. R. M., Toscano, A., Aguennouz, M., Martini, D., Polizzi, E., Ranieri-Raggi, M., Moir, A. J. G., Migliorato, A., Musumeci, O., Vita, G., & Raggi, A. (2006). Immunohistochemical analysis of human skeletal muscle AMP deaminase deficiency. Evidence of a correlation between the muscle HPRG content and the level of the residual AMP deaminase activity. Journal of Muscle Research and Cell Motility, 27(1), 83-92. https://doi.org/10.1007/s10974-006-9059-4