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Articles by M Hirano
Total Records ( 2 ) for M Hirano
  R Horvath , J. P Kemp , H. A. L Tuppen , G Hudson , A Oldfors , S. K. N Marie , A. R Moslemi , S Servidei , E Holme , S Shanske , G Kollberg , P Jayakar , A Pyle , H. M Marks , E Holinski Feder , M Scavina , M. C Walter , J Coku , A Gunther Scholz , P. M Smith , R McFarland , Z. M. A Chrzanowska Lightowlers , R. N Lightowlers , M Hirano , H Lochmuller , R. W Taylor , P. F Chinnery , M Tulinius and S. DiMauro

Childhood-onset mitochondrial encephalomyopathies are usually severe, relentlessly progressive conditions that have a fatal outcome. However, a puzzling infantile disorder, long known as ‘benign cytochrome c oxidase deficiency myopathy’ is an exception because it shows spontaneous recovery if infants survive the first months of life. Current investigations cannot distinguish those with a good prognosis from those with terminal disease, making it very difficult to decide when to continue intensive supportive care. Here we define the principal molecular basis of the disorder by identifying a maternally inherited, homoplasmic m.14674T>C mt-tRNAGlu mutation in 17 patients from 12 families. Our results provide functional evidence for the pathogenicity of the mutation and show that tissue-specific mechanisms downstream of tRNAGlu may explain the spontaneous recovery. This study provides the rationale for a simple genetic test to identify infants with mitochondrial myopathy and good prognosis.

  M Zhou , H. J He , M Hirano , M Sekiguchi , O Tanaka , K Kawahara and H. Abe

ATP-sensitive K+ (KATP) channel subunits were investigated in rat submandibular gland (SMG). RT-PCR detected the presence of mRNA transcripts of the Kir6.1, Kir6.2, SUR2A, and SUR2B in the SMG, whereas SUR1 mRNA was barely detected. Western blot analysis provided the evidence that these four KATP channel subunits are expressed in rat SMG. Immunostaining detected that these four KATP channel subunits are widely distributed, with different intensities, in myoepithelial cells, epithelial cells of intercalated ducts, granular convoluted tubules, striated ducts, and excretory ducts. Immunofluorescence double staining showed that Kir6.1 and Kir6.2 colocalized with SUR2A in the myoepithelial cells, granular convoluted tubules, striated ducts, and excretory ducts. Kir6.1 and Kir6.2 also colocalized with SUR2B, mainly in the duct system, e.g., the granular convoluted tubules, striated ducts, and excretory ducts. Taken together, these results indicate that the KATP channels in SMG may consist of Kir6.1, Kir6.2, SUR2A, and SUR2B, with various combinations of colocalization with each other, and may play important roles in rat SMG during salivary secretion. (J Histochem Cytochem 58:499–507, 2010)

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