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Mitochondrial DNA depletion and fatal infantile hepatic failure due to mutations in the mitochondrial polymerase γ (POLG) gene: a combined morphological/enzyme histochemical and immunocytochemical/biochemical and molecular genetic study.

Müller-Höcker J, Horvath R, Schäfer S, Hessel H, Müller-Felber W, Kühr J, Copeland WC, Seibel P - J. Cell. Mol. Med. (2011)

Bottom Line: The pathological characterization of the tissues revealed a severe depletion of mtDNA (mitochondrial DNA) that was most pronounced in liver, followed by a less severe, but still significant depletion in skeletal muscle and the heart.A variable defect pattern was found in liver, muscle and heart tissue as revealed by biochemical, cytochemical, immunocytochemical and in situ hybridization analysis.Their disappearance could also aggravate the mtDNA depletion and contribute to the non-homogenous defect pattern.

View Article: PubMed Central - PubMed

Affiliation: Pathologisches Institut der Ludwig-Maximilians-Universität, München, Germany. josef.mueller-hoecker@med.uni-muenchen.de

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Cytochrome-c-oxidase immunohistochemistry in the liver. (A) The subunit II/III lacks in most of the hepatocytes but is retained in the biliary ducts (↑). (B) Subunit Vab. A mosaic defect pattern is seen with coexisting deficient and reacting hepatocytes. The bile duct epithelium reacts normally (↑). Bar A, B: 50 μM.
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fig04: Cytochrome-c-oxidase immunohistochemistry in the liver. (A) The subunit II/III lacks in most of the hepatocytes but is retained in the biliary ducts (↑). (B) Subunit Vab. A mosaic defect pattern is seen with coexisting deficient and reacting hepatocytes. The bile duct epithelium reacts normally (↑). Bar A, B: 50 μM.

Mentions: Immunohistochemistry disclosed a severe loss of cytochrome-c-oxidase subunits II/III, Vab, sparing small islands of hepatocytes. In contrast, the bile ducts reacted normally (Fig. 4A, B).


Mitochondrial DNA depletion and fatal infantile hepatic failure due to mutations in the mitochondrial polymerase γ (POLG) gene: a combined morphological/enzyme histochemical and immunocytochemical/biochemical and molecular genetic study.

Müller-Höcker J, Horvath R, Schäfer S, Hessel H, Müller-Felber W, Kühr J, Copeland WC, Seibel P - J. Cell. Mol. Med. (2011)

Cytochrome-c-oxidase immunohistochemistry in the liver. (A) The subunit II/III lacks in most of the hepatocytes but is retained in the biliary ducts (↑). (B) Subunit Vab. A mosaic defect pattern is seen with coexisting deficient and reacting hepatocytes. The bile duct epithelium reacts normally (↑). Bar A, B: 50 μM.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3822808&req=5

fig04: Cytochrome-c-oxidase immunohistochemistry in the liver. (A) The subunit II/III lacks in most of the hepatocytes but is retained in the biliary ducts (↑). (B) Subunit Vab. A mosaic defect pattern is seen with coexisting deficient and reacting hepatocytes. The bile duct epithelium reacts normally (↑). Bar A, B: 50 μM.
Mentions: Immunohistochemistry disclosed a severe loss of cytochrome-c-oxidase subunits II/III, Vab, sparing small islands of hepatocytes. In contrast, the bile ducts reacted normally (Fig. 4A, B).

Bottom Line: The pathological characterization of the tissues revealed a severe depletion of mtDNA (mitochondrial DNA) that was most pronounced in liver, followed by a less severe, but still significant depletion in skeletal muscle and the heart.A variable defect pattern was found in liver, muscle and heart tissue as revealed by biochemical, cytochemical, immunocytochemical and in situ hybridization analysis.Their disappearance could also aggravate the mtDNA depletion and contribute to the non-homogenous defect pattern.

View Article: PubMed Central - PubMed

Affiliation: Pathologisches Institut der Ludwig-Maximilians-Universität, München, Germany. josef.mueller-hoecker@med.uni-muenchen.de

Show MeSH
Related in: MedlinePlus