Results: 5

1.
Figure 1

Figure 1. From: Autophagy and Mitochondria in Pompe Disease: Nothing is so New as What Has Long Been Forgotten*.

Convergence of endocytic and autophagic pathways

Nina Raben, et al. Am J Med Genet C Semin Med Genet. ;160(1):13-21.
2.
Figure 4

Figure 4. From: Autophagy and Mitochondria in Pompe Disease: Nothing is so New as What Has Long Been Forgotten*.

Autophagic area in fibers from an untreated 5-year-old Pompe patient. Top: the fiber was stained for LAMP (red) and LC3 (green). Nuclei are shown in white. Bottom: unstained fixed fibers observed by phase contrast transmitted microscopy.

Nina Raben, et al. Am J Med Genet C Semin Med Genet. ;160(1):13-21.
3.
Figure 3

Figure 3. From: Autophagy and Mitochondria in Pompe Disease: Nothing is so New as What Has Long Been Forgotten*.

Acid α-glucosidase is responsible for the break-down of glycogen in the lysosomes. When the enzyme is absent or deficient, glycogen accumulates in the lysosomes. It is not clear how glycogen is transported from the cytoplasm to the lysosomes. If this transport involves the delivery of glycogen in the autophagosomes, then suppression of autophagy would reduce the traffic and decrease the amount of lysosomal glycogen. The degradation of the cytoplasmic glycogen would proceed unaffected.

Nina Raben, et al. Am J Med Genet C Semin Med Genet. ;160(1):13-21.
4.
Figure 2

Figure 2. From: Autophagy and Mitochondria in Pompe Disease: Nothing is so New as What Has Long Been Forgotten*.

Autophagy in skeletal muscle of Pompe mice. Top: electron micrographs of type II-rich muscle (psoas) from a 5 month-old Pompe mouse showing autophagic vacuoles. Bottom: autophagic buildup in the core of a fiber derived from psoas muscle. The fiber is stained for lysosomal marker LAMP (green) and autophagosomal marker LC3 (red). Nuclei are shown in white. Bars: 0.5 microns for EM and 10 microns for the stained fiber.

Nina Raben, et al. Am J Med Genet C Semin Med Genet. ;160(1):13-21.
5.
Figure 5

Figure 5. From: Autophagy and Mitochondria in Pompe Disease: Nothing is so New as What Has Long Been Forgotten*.

Some of the mitochondria in muscles of Pompe mice and autophagy-deficient Pompe mice contain glycogen inclusions (white arrowheads). Occasionally, a single mitochondrion may contain several inclusions (not shown). In the example in the top panel, glycogen occupies a limited portion of the mitochondrial space; the mitochondrial cristae (black arrows) have the usual transverse orientation in areas away from the inclusion. In the bottom panel, the inclusion fills a larger portion of the mitochondrion; all visible cristae appear reoriented and abnormal around the inclusion. Glycogen inclusions thus affect the internal structure and, possibly, the function of mitochondria. Bars: 500 nm (top) and 100 nm (bottom).

Nina Raben, et al. Am J Med Genet C Semin Med Genet. ;160(1):13-21.

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