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1.
Figure 13

Figure 13. From: Mitochondrial mosaics in the liver of 3 infants with mtDNA defects.

COX reaction: Single reacting semicircular crista. Other mitochondria are (nearly) unreactive; two show invaginations (arrow) containing glycogenrosettes and cytosol. Patient 3.

Frank Roels, et al. BMC Clin Pathol. 2009;9:4-4.
2.
Figure 15

Figure 15. From: Mitochondrial mosaics in the liver of 3 infants with mtDNA defects.

COX reaction: strongly reactive mitochondria in a cell identified by its microfilaments as vascular smooth muscle. Patient 1.

Frank Roels, et al. BMC Clin Pathol. 2009;9:4-4.
3.
Figure 9

Figure 9. From: Mitochondrial mosaics in the liver of 3 infants with mtDNA defects.

COX reaction: parenchymal cell in the center shows mitochondria devoid of reaction product. It is surrounded by cells with well-stained mitochondria. Tiny granules are glycogen. Patient 2.

Frank Roels, et al. BMC Clin Pathol. 2009;9:4-4.
4.
Figure 11

Figure 11. From: Mitochondrial mosaics in the liver of 3 infants with mtDNA defects.

COX reaction: only single or few cristae show COX reaction product. Large lipid globules are either dark, grey or largely dissolved. Tiny dark granules are glycogen. Patient 3.

Frank Roels, et al. BMC Clin Pathol. 2009;9:4-4.
5.
Figure 10

Figure 10. From: Mitochondrial mosaics in the liver of 3 infants with mtDNA defects.

COX reaction: 2 hepatocytes with well-contrasted mitochondrial envelopes and cristae. In the third cell that contains many lipid globules, only some mitochondria have a few stained cristae (arrow); mitochondrial numbers appear increased. Patient 2.

Frank Roels, et al. BMC Clin Pathol. 2009;9:4-4.
6.
Figure 16

Figure 16. From: Mitochondrial mosaics in the liver of 3 infants with mtDNA defects.

Immunocytochemistry of OXPHOS proteins in liver of patient 3. 16a: complex IV (coloured red) is present only in islets of cells amid unstained parenchyma. 16b: complex II is present in red granules, i.e. in mitochondria in all parenchymal cells.

Frank Roels, et al. BMC Clin Pathol. 2009;9:4-4.
7.
Figure 12

Figure 12. From: Mitochondrial mosaics in the liver of 3 infants with mtDNA defects.

COX reaction: mitochondrion (asterisk) with only two circular cristae reacting; other mitochondria in this hepatocyte are unstained. Adjacent cell shows strongly reacting mitochondrial envelopes and cristae; also lipid. Dark bodies are peroxisomes (arrows). Patient 1.

Frank Roels, et al. BMC Clin Pathol. 2009;9:4-4.
8.
Figure 14

Figure 14. From: Mitochondrial mosaics in the liver of 3 infants with mtDNA defects.

COX reaction in non-parenchymal cells. COX reaction: numerous well stained mitochondria in lining cells of a bile duct (or capillary); a basal lamina is present. Adjacent hepatic parenchyma shows dark peroxisomes while mitochondria have only a few reactive cristae. Patient 1.

Frank Roels, et al. BMC Clin Pathol. 2009;9:4-4.
9.
Figure 5

Figure 5. From: Mitochondrial mosaics in the liver of 3 infants with mtDNA defects.

Liver, patient 3. 5a: micronodular cirrhosis at 18 months; trichrome stain. 5b: small group of COX reactive parenchymal cells adjacent to an unstained region. Cryostat section, cytochrome oxidase reaction, nuclei slightly counterstained with methylgreen.

Frank Roels, et al. BMC Clin Pathol. 2009;9:4-4.
10.
Figure 8

Figure 8. From: Mitochondrial mosaics in the liver of 3 infants with mtDNA defects.

Liver: ultrastructure and reaction for cytochrome oxidase. Fig 8: COX reaction product stains mitochondrial envelope and cristae. 4 parenchymal cells have different levels of mitochondrial reaction. Peroxisomes are dark round bodies (arrows), they stand out in the hepatocyte without COX reaction; here mitochondrial numbers appear increased. Patient 1.

Frank Roels, et al. BMC Clin Pathol. 2009;9:4-4.
11.
Figure 7

Figure 7. From: Mitochondrial mosaics in the liver of 3 infants with mtDNA defects.

Liver, patient 2, cytochrome oxidase stain. 7a: Different levels of mitochondrial staining in adjacent hepatocytes, cryostat section. Nuclei slightly counterstained with methylgreen reveal cells without staining (arrow). 7b: Higher magnification: COX-positive parenchymal cells alternate with unstained ones. Large dark structures are erythrocytes (arrow). Nuclei slightly counterstained with methylgreen, cryostat section.

Frank Roels, et al. BMC Clin Pathol. 2009;9:4-4.
12.
Figure 2

Figure 2. From: Mitochondrial mosaics in the liver of 3 infants with mtDNA defects.

Patient 2, brain MRI. 2a: T2 weighted; mild tot moderate dilatation of ventricles and peripheral liquor spaces suggesting cerebral atrophy. 2b: TRACE sequence shows cytotoxic oedema in cortex of right central gyrus. 2c: 3 months later. Focal cortical atrophy of central gyrus bilaterally. 2d: 2 years later. Focal cortical atrophy of central gyrus bilaterally.

Frank Roels, et al. BMC Clin Pathol. 2009;9:4-4.
13.
Figure 3

Figure 3. From: Mitochondrial mosaics in the liver of 3 infants with mtDNA defects.

Muscle pathology. 3a: ATP-ase reaction at pH 4.6 shows regular sizes and normal chessboard distribution of 3 fiber types. Patient 2. 3b: Gomori trichrome shows normal image; absence of ragged-red fibers. Patient 1. 3c: Cytochrome oxidase activity shows normal distribution of mitochondria in distinct fiber types; compare to 3d. Patient 3. 3d: NADH-tetrazolium reductase shows normal distribution of mitochondria; compare to COX stain in 3c. Patient 1.

Frank Roels, et al. BMC Clin Pathol. 2009;9:4-4.
14.
Figure 1

Figure 1. From: Mitochondrial mosaics in the liver of 3 infants with mtDNA defects.

Patient 1, brain MRI. 1a: TRACE sequence shows cytotoxic oedema in frontoparietal white matter bilaterally, and in right occipital. 1b: FLAIR sequence, right occipital and bilateral frontoparietal lesions; compare to fig 1a. 1c: 5 months later. T1 weighted without iv contrast. Bifrontal white matter loss with lacunae, small lacuna in right occipital. 1d: Coronal section; bilateral loss of white matter; lacunae lined by high signal interpreted as gliosis.

Frank Roels, et al. BMC Clin Pathol. 2009;9:4-4.
15.
Figure 6

Figure 6. From: Mitochondrial mosaics in the liver of 3 infants with mtDNA defects.

Liver, patient 1, cytochrome oxidase stain. 6a: low power view of cryostat section; islet of stained cells surrounded by unstained parenchyma. Large dark granules are erythrocytes in sinusoids and Kupffer cells that stain for peroxidase (arrow). Patient 1. 6b: high power of sharp border between COX stained and unstained parenchymal cells. Numerous tightly packed granules are mitochondria. In the unstained cells, the fewer small round granules are peroxisomes reacting by their catalase. Postosmicated, semithin plastic section.

Frank Roels, et al. BMC Clin Pathol. 2009;9:4-4.
16.
Figure 4

Figure 4. From: Mitochondrial mosaics in the liver of 3 infants with mtDNA defects.

Blue native PAGE of the OXPHOS complexes. In-gel activity staining. In patient 1 (Pearson syndrome), the activity of complex IV is severely decreased in liver. In patient 2 (POLG mutation), complex I and complex II are slightly decreased in skeletal muscle (a limited amount of muscle from the patient was available for testing and the complex II band was weak in the control as well). Normal activities are detected in mitochondria from skeletal muscle of patient 3 (POLG mutation). In liver mitochondria of this patient the activities of complex I, III and IV are slightly decreased. In mitochondria from all patients subcomplexes of complex V are seen (only weakly in patient 2), which is considered as a marker of disturbed intramitochondrial protein synthesis.

Frank Roels, et al. BMC Clin Pathol. 2009;9:4-4.

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