Developmental Expression of Sarcoglycan Gene Products in Cultured Myocytes

Noguchi, S.; Wakabayashi, Eriko; Imamura, Michihiro; Yoshida, Motoaki; Ozawa, Eijiro

doi:10.1006/bbrc.1999.1163

Cited by 35 publications

(44 citation statements)

References 23 publications

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“…In our experiments, it is unclear to what degree donor-derived nuclei have entered into the myogenic program. For example, dystrophin and sarcoglycan are expressed late in myotube differentiation in cell culture models (39), while MyoD and desmin serve as early markers of myoblast status. Because sarcoglycan is a late marker of myogenic specification, it is possible that stem cell-derived nuclei have partially differentiated into a myocyte lineage.…”

Section: Discussionmentioning

confidence: 99%

Transplanted hematopoietic stem cells demonstrate impaired sarcoglycan expression after engraftment into cardiac and skeletal muscle

Lapidos¹,

Chen²,

Earley³

et al. 2004

J. Clin. Invest.

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Pluripotent bone marrow-derived side population (BM-SP) stem cells have been shown to repopulate the hematopoietic system and to contribute to skeletal and cardiac muscle regeneration after transplantation. We tested BM-SP cells for their ability to regenerate heart and skeletal muscle using a model of cardiomyopathy and muscular dystrophy that lacks δ-sarcoglycan. The absence of δ-sarcoglycan produces microinfarcts in heart and skeletal muscle that should recruit regenerative stem cells. Additionally, sarcoglycan expression after transplantation should mark successful stem cell maturation into cardiac and skeletal muscle lineages. BM-SP cells from normal male mice were transplanted into female δ-sarcoglycan-null mice. We detected engraftment of donor-derived stem cells into skeletal muscle, with the majority of donor-derived cells incorporated within myofibers. In the heart, donor-derived nuclei were detected inside cardiomyocytes. Skeletal muscle myofibers containing donor-derived nuclei generally failed to express sarcoglycan, with only 2 sarcoglycan-positive fibers detected in the quadriceps muscle from all 14 mice analyzed. Moreover, all cardiomyocytes with donor-derived nuclei were sarcoglycan-negative. The absence of sarcoglycan expression in cardiomyocytes and skeletal myofibers after transplantation indicates impaired differentiation and/or maturation of bone marrow-derived stem cells. The inability of BM-SP cells to express this protein severely limits their utility for cardiac and skeletal muscle regeneration.

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Section: Discussionmentioning

confidence: 99%

Transplanted hematopoietic stem cells demonstrate impaired sarcoglycan expression after engraftment into cardiac and skeletal muscle

Lapidos¹,

Chen²,

Earley³

et al. 2004

J. Clin. Invest.

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“…Morris, Wolfson Centre for Inherited Neuromuscular Disease), 26 NCL-DYSB recognizing amino acids 321 to 494 (Novocastra Laboratories, Newcastle, UK), NCL-DYS1 recognizing amino acids 1181 to 1388 (Novocastra), MANDYS8 recognizing amino acids 1431 to 1505 (Sigma-Aldrich, St Louis, Mo), F22.9C5 recognizing amino acids 1840 to 2266 (Alexis, Lausen, Switzerland), or NCL-DYS2 recognizing amino acids 3669 to 3685 (Novocastra); mouse monoclonal antibodies against ␤-dystroglycan (NCL-b-DG, Novocastra), ␤-sarcoglycan (NCL-b-SG, Novocastra), ␥-sarcoglycan (NCL-g-SG, Novocastra), or ␦-sarcoglycan (DSG-1) 27 ; rabbit polyclonal antibodies against utrophin (UT-2), 28 ␣-sarcoglycan (␣-SG2), 29 or -calpain (anticalpain 1 large subunit domain IV; Sigma-Aldrich). The primary antibodies were labeled with fluorescein-conjugated goat anti-mouse or anti-rabbit immunoglobulin G (Molecular Probes, Eugene, Ore), and signals were recorded photographically with a confocal laser scanning microscope (TCSSP, Leica).…”

Section: Immunohistochemistrymentioning

confidence: 99%

Selective Vacuolar Degeneration in Dystrophin-Deficient Canine Purkinje Fibers Despite Preservation of Dystrophin-Associated Proteins With Overexpression of Dp71

et al. 2008

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Background-Respiratory support therapy significantly improves life span in patients with Duchenne muscular dystrophy; cardiac-related fatalities, including lethal arrhythmias, then become a crucial issue. It is therefore important to more thoroughly understand cardiac involvement, especially pathology of the conduction system, in the larger Duchenne muscular dystrophy animal models such as dystrophic dogs. Methods and Results-When 10 dogs with canine X-linked muscular dystrophy in Japan (CXMD J ) were examined at the age of 1 to 13 months, dystrophic changes of the ventricular myocardium were not evident; however, Purkinje fibers showed remarkable vacuolar degeneration as early as 4 months of age. The degeneration of CXMD J Purkinje fibers was coincident with overexpression of Dp71 at the sarcolemma and translocation of -calpain to the cell periphery near the sarcolemma or in the vacuoles. Immunoblotting of the microdissected fraction showed that -calpain-sensitive proteins such as desmin and cardiac troponin-I or -T were selectively degraded in the CXMD J Purkinje fibers. Utrophin was highly upregulated in the earlier stage of CXMD J Purkinje fibers, but the expression was dislocated when vacuolar degeneration was recognized at 4 months of age. Nevertheless, the expression of dystrophin-associated proteins ␣-, ␤-, ␥-, and ␦-sarcoglycans and ␤-dystroglycan was well maintained at the sarcolemma of Purkinje fibers. Conclusions-Selective vacuolar degeneration of Purkinje fibers was found in the early stages of dystrophin deficiency.Dislocation of utrophin besides upregulation of Dp71 can be involved with this pathology. The degeneration of Purkinje fibers can be associated with the distinct deep Q waves in ECG and fatal arrhythmia seen in dystrophin deficiency.

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“…We used a C2/4 cell line, a subclone of C2C12 mesenchymal cells, which retains only myogenic potency (43,44). Because C2/4 cells express Notch1 (see Fig.…”

Section: Fig 5 Nov Notch1mentioning

confidence: 99%

The Nephroblastoma Overexpressed Gene (NOV/ccn3) Protein Associates with Notch1 Extracellular Domain and Inhibits Myoblast Differentiation via Notch Signaling Pathway

Sakamoto¹,

Yamaguchi²,

Ando³

et al. 2002

Journal of Biological Chemistry

179

157

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Developmental Expression of Sarcoglycan Gene Products in Cultured Myocytes

Cited by 35 publications

References 23 publications

Transplanted hematopoietic stem cells demonstrate impaired sarcoglycan expression after engraftment into cardiac and skeletal muscle

Transplanted hematopoietic stem cells demonstrate impaired sarcoglycan expression after engraftment into cardiac and skeletal muscle

Selective Vacuolar Degeneration in Dystrophin-Deficient Canine Purkinje Fibers Despite Preservation of Dystrophin-Associated Proteins With Overexpression of Dp71

The Nephroblastoma Overexpressed Gene (NOV/ccn3) Protein Associates with Notch1 Extracellular Domain and Inhibits Myoblast Differentiation via Notch Signaling Pathway

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