Statistically Significant Differences in the Number of CD24 Positive Muscle Fibers and Satellite Cells between Sarcoglycanopathy and Age-Matched Becker Muscular Dystrophy Patients.

Abstract

Object: The aim of this study was to reveal variations in the patterns of expression of the cell surface proteins in regenerating fibers and those in the number of satellite cells to gain an understanding of the pathological processes involved in sarcoglycanopathy. Methods: Wehave reported that there is a reduction of the beta-1 subunit of laminin, heparan sulfate proteoglycan (HSPG), and HCAM(CD44) in Japanese patients with sarcoglycanopathy.Here, we investigated immunohistochemically the expression of the neural cell adhesion molecule (NCAM),which is a marker for humanregenerating muscle and satellite cell, and CD24, which appears to be expressed in the early stages of the regeneration process. Patients: Weinvestigated six Japanese patients with sarcoglycanopathy, and compared to age-matched Becker muscular dystrophy.Results: Wefound that the incidences of muscle fibers with increased NCAM were not statistically different between the two groups. However, the incidences of muscle fibers with increased CD24and those of NCAM positive satellite cells were very low in sarcoglycanopathy and were statistically different between sarcoglycanopathy and age-matched Becker muscular dystrophies. Conclusion: The poor expression of CD24and the fewer satellite cells in sarcoglycanopathy without significant difference in the number of total regenerating fibers suggest that a different regeneration process is involved in sarcoglycanopathy compared to that in other types of muscular dystrophy. (Interal Medicine 38: 412-415, 1999)

ANKK1 is found in myogenic precursors and muscle fibers subtypes with glycolytic metabolism

“…From a genetic point of view, the ANKK1 gene location in the NTAD genomic cluster where the NCAM1 gene maps also supports a function of this locus upon myogenesis. In addition to the well-established role of NCAM1 in neurogenesis, this protein has also been identified in embryonic myoblasts [ 42 ], adult SCs [ 43 ] and regenerating fibers in different neuromuscular pathologies [ 44 – 46 ]. Therefore, the findings of ANKK1 and NCAM1 in embryonic and adult myogenesis extend the role of the NTAD cluster beyond neurodevelopment to other cell lineages during development.…”

Cause of Progression in Duchenne Muscular Dystrophy: Impaired Differentiation More Probable Than Replicative Aging

“…Defects of muscle regeneration are not necessarily identical in DMD and DMD-like sarcoglycanopathies. Indeed, the latter differ from dystrophinopathies in several factors which may influence regeneration such as dystrophin expression itself, TGF-b1 expression [77], or satellite cell numbers and markers [25]. Thus, beyond the genetic defects, secondary causes of progression in DMD and DMD-like dystrophies may also diverge.…”

Amplification of the CD24 Gene Is an Independent Predictor for Poor Prognosis of Breast Cancer

“…CD24 is located on chromosome 6q21 and encodes a glycosylphosphatidylinositol-linked cell surface glycoprotein (Hough et al, 1994). It is expressed on hematopoietic cells (Li et al, 2004; Israel et al, 2005), neural cells (Nielsen and Cohen, 1996), epithelial cells (Sleeman et al, 2006), muscle cells (Higuchi et al, 1999), stem cells (Lawson et al, 2006; Shackleton et al, 2006), and many other cell types including cancer cells (Kristiansen et al, 2002; Fillmore and Kuperwasser, 2007; Sagiv et al, 2008). In addition to the immunological functions (Li et al, 2004, 2006; Liu and Zheng, 2007), recent studies have implicated CD24 function in tumorigenesis and progression of multiple cancer types, including carcinomas in lung, prostate, ovarian, breast, and brain (Kristiansen et al, 2002, 2003; Fillmore and Kuperwasser, 2007; Sagiv et al, 2008).…”