Localization and amount of myoglobin and myoglobin mRNA in ragged-red fiber of patients with mitochondrial encephalomyopathy

✍ Scribed by Makoto Kunishige; Takao Mitsui; Masashi Akaike; Masayuki Shono; Hisaomi Kawai; Shiro Saito

• Publisher: John Wiley and Sons
• Year: 1996
• Tongue: English
• Weight: 890 KB
• Volume: 19
• Category: Article
• ISSN: 0148-639X
• DOI: 10.1002/(sici)1097-4598(199602)19:2<175::aid-mus8>3.0.co;2-b

✦ Synopsis

The localization and amounts of rnyoglobin (Mb) and Mb rnRNA in ragged-red fibers (RRF) in skeletal muscle of 6 patients with rnitochondrial encephalomyopathy were examined imrnunohistochemically and by in situ hybridization. The amounts of Mb and Mb mRNA were expressed in terms of optical densities (ODs) of Mb immunostaining and Mb mRNA signals. In nonatrophic RRF, Mb was predominantly seen in the ragged-red region and Mb mRNA signals were increased throughout the sarcoplasm. The amounts of Mb and Mb mRNA in nonatrophic RRF were greater than those in nonatrophic non-RRF. In contrast, the localization and amount in atrophic RRF were similar to those in atrophic non-RRF. Thus, Mb synthesis in nonatrophic RRF may increase to compensate for mitochondrial dysfunction and to supply sufficient oxygen to mitochondria, but this compensatory function may be impaired in atrophic RRF.