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頁籤選單縮合
題 名 | Increase of Mitochondria Number in Muscle Fibers of Limb-Girdle Dystrophy (LGD)=Limb-Girdle Dystrophy患者之肌纖維中粒線體增生情形之探討 |
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作 者 | 許燿東; 思葛爾; 張民基; 曹汶龍; | 書刊名 | 醫學研究 |
卷 期 | 16:2 1995.09[民84.09] |
頁 次 | 頁83-101 |
分類號 | 415.9413 |
關鍵詞 | 肢端帶漸進性肌肉萎縮症; 粒線體; 電子顯微鏡; Limb-girdle muscular dystrophy; Mitochondria; Ultrastructure; Dystrophin; |
語 文 | 英文(English) |
中文摘要 | 以電子顯微鏡檢察分析肢端帶肌肉漸進性萎縮症(LGD)的非壞死性肌細胞的研究,。共11病例(內含3位男性,8位女性,年齡為15至69歲,發病時間從3至46年不等)及10位正常對照組肌肉組織。所有病人的肌肉切片都經過anti-dystrophine抗體染色及Western blot篩檢鑑定為非dystrophin病變。發現在LGD的粒腺體總面積佔全肌肉細胞面積的6.6±0.69%,而對照組為3.29±0.52%,其p值小於0.001。這種粒腺體的增加主要為數目的增加而大部份的粒腺體形態學為正常。當然除了粒腺體量的增加外尚有一些形態學的變化,如trnasverse tubular (t)系統的變化。整個胞膜並無缺陷。由胞膜的正常與粒腺體的增加兩方面都與Duchenne肌肉進行性萎縮症所觀察到的胞膜缺陷及粒腺體減少完全不同的兩種變化。這種粒腺體增加的現象似乎反應出LGD這個疾病可能與粒腺體的代謝出問題有密切的關聯。或是因其他的病理機轉刺激粒腺體的複製增加所致。 |
英文摘要 | Ultrastructures of nonnecrotic muscle fibers from limb-gridle dystrophy (LGD) were investigated. Eleven LGD patients (3 men, 8 women, ages 15 to 69, symptoms identified for 3 to 46 years) and 10 nonweak controls were studied. Dystrophinopathy was excluded by immunostaining and western blotting in all patients. The mitochondrial fraction of the fiber volume (mean ± SD) was 6.6±0.69% in LGD, and 3.29±0.52% in the controls (p<0.001). The increase was due to an increased number of mitochondria; most individual mitochondria appear normal. In addition, qualitative abnormalities of the transverse tubular (t) system were found in many fibers. No sarcolemmal defect was detected. The findings differ from those in Duchenne dystrophy of which nonnecrotic fibers display sarcolemmal defects, but show decrease in mitochondrial area. The consistent increase of mitochondria in LGD points to a possible primary defect of mitochondrial function (energy generation), or secondary energy demand that stimulates mitochondrial proliferation. |
本系統中英文摘要資訊取自各篇刊載內容。