지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2017.1
- 수록면
- 359 - 365 (7page)
이용수
초록· 키워드
오류제보하기
Background and Purpose The pathogenesis of mitochondrial disease (MD) involves the disruption of cellular energy metabolism, which results from defects in the mitochondrial respiratory chain complex (MRC). We investigated whether infants with MRC I defects showed ultrastructural changes in skeletal muscle. Methods Twelve infants were enrolled in this study. They were initially evaluated for unexplained neurodegenerative symptoms, myopathies, or other progressive multiorgan involvement, and underwent muscle biopsies when MD was suspected. Muscle tissue samples were subjected to biochemical enzyme assays and observation by transmission electron microscopy. We compared and analyzed the ultrastructure of skeletal muscle tissues obtained from patients with and without MRC I defects. Results Biochemical enzyme assays confirmed the presence of MRC I defects in 7 of the 12 patients. Larger mitochondria, lipid droplets, and fused structures between the outer mitochondrial membrane and lipid droplets were observed in the skeletal muscles of patients with MRC I defects. Conclusions Mitochondrial functional defects in MRC I disrupt certain activities related to adenosine triphosphate synthesis that produce changes in the skeletal muscle. The ultrastructural changes observed in the infants in this study might serve as unique markers for the detection of MD.
목차
등록된 정보가 없습니다.
참고문헌 (34)
참고문헌 신청
Acehan D / 2007 / Comparison of lymphoblast mitochondria from normal subjects and patients with Barth syndrome using electron microscopic tomography / Lab Invest 87:40~48
Mannella CA / 2006 / Structure and dynamics of the mitochondrial inner membrane cristae / Biochim Biophys Acta 1763:542~548
Scorrano L / 2002 / A distinct pathway remodels mitochondrial cristae and mobilizes cytochrome c during apoptosis / Dev Cell 2:55~67
Ding WX / 2012 / Electron microscopic analysis of a spherical mitochondrial structure / J Biol Chem 287:42373~42378
Mannella CA / 2008 / Structural diversity of mitochondria: functional implications / Ann N Y Acad Sci 1147:171~179
Mannella CA / 2006 / Structure and dynamics of the mitochondrial inner membrane cristae / Biochim Biophys Acta 1763:542~548
Scorrano L / 2002 / A distinct pathway remodels mitochondrial cristae and mobilizes cytochrome c during apoptosis / Dev Cell 2:55~67
Ding WX / 2012 / Electron microscopic analysis of a spherical mitochondrial structure / J Biol Chem 287:42373~42378
Mannella CA / 2008 / Structural diversity of mitochondria: functional implications / Ann N Y Acad Sci 1147:171~179
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