English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Global: Zendy Tools annual

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the summarisation as premium feature

Summarisation

Insights

Presents the pdf analysis as premium feature

PDF Analysis

Presents the zaia usage as premium feature

ZAIA

Global: Zendy Tools monthly

Global: Zendy Plus monthly

Presents the access of premium content as premium feature

Premium Content

Global: Zendy Plus annual

Global: Zendy Free Plan

Mitochondria from the muscle of a patient with mitochondrial myopathy contained paracrystalline inclusion bodies. To characterize the mitochondrial fraction containing the crystalline materials, the muscle homogenate was subjected to a Eicoll density gradient centrifugation. The mitochondrial fraction was identified by immunoblotting with specific antisera against the F,-ATPase ,8-subunit and lipoamide acetyltransferase of the pyruvate dehydrogenase complex. The mitochondria from the patient had a density higher than in mitochondria from controls. This method is useful for isolating abnormal mitochondria from muscle specimens of about 0.1 g. Paracrystalline inclusion bodies have often been observed in mitochondria from muscles of patients with mitochondrial myopathy (14, l5). These crystalloids in diseased human muscle have been described (l, 16), but few information is available on their significance in the pathogenesis of the mitochondrial myopathies. To characterize these inclusion bodies, we first attempted to isolate abnormal mitochondria by density gradient centrifugation. Mitochondria from muscle of a patient with mitochondrial myopathy had a density higher than that of mitochondria from control muscle. MATERIALS AND METHODS Case The patient was a 54-year-old Japanese female with progressive ophthalmoplegia and generalized muscle weakness during the last ten years. In l984 at the age of 53, she had an episode of apnea requiring hospitalization. When she was admitted to the Hospital of Jichi Medical School, laboratory data on electrolytes, and creatine phosphokinase and aldolase activities were normal. The concentration of lactate and pyruvate was elevated in blood and cerebrospinal fluid. A brain CT scan showed a low density in the periventricular region and diffuse cortical atrophy. Retinal and cardiac involvement was absent. She died of respiratory failure at the age of54 in 1985. One of her brothers and one of her two sons have been diagnosed as the same disease. In addition, her grandmother, mother, three brothers, one sister and four cousins had symptoms of the same disease. Thus the family history suggested autosomal dominant inheritance or maternal inheritance. Muscle With the family’s consent, a specimen was

<b>INCREASED DENSITY OF MITOCHONDRIA ISOLATED FROM THE SKELETAL MUSCLE OF A PATIENT WITH MITOCHONDRIAL </b><b>MYOPATHY </b>