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Utrophin is a submembranous protein structurally similar to dystrophin and is widely expressed, albeit at low levels, in the sarcolemma Extracellular proteins, collagen IV, perlecan, fibronectin, agrin, and laminin, are the main components of the basal lamina.

Collagen VI is present in the interstitium but is associated directly with collagen IV We observed very little staining of only collagens IV and VI in vacuolar membranes, indicating that the membranes hardly contain these collagens.

Based on our findings, we deduce that the vacuolar membrane of AVSFs in Danon disease and related AVMs have most of the sarcolemmal proteins ranging from cytoplasmic dystrophin to the extracellular laminin.

Nevertheless, they are distinct from motor endplates because the membranes lacked AChRs. In the early stages of formation of the neuromuscular junction, AChE and AChRs are localized diffusely throughout the sarcolemma.

When axon terminals make contact with muscle cells, postjunctional folds are quickly formed. These facts support our hypothesis that the vacuoles are intracellular enclosed spaces, because, if AVSF were derived from sarcolemma, then AChE-expressing vacuoles should be located near neuromuscular junctions rather than scattered in the cytoplasm.

Furthermore, the presence of AChE without AChRs clearly indicates that the vacuolar membranes are distinct from either junctional or extra-junctional sarcolemma and suggests that they are formed through a unique process.

Most autophagic vacuoles in Danon disease are autolysosomes rather than autophagosomes, which lack enzymatic activity. These are indicated by the demonstration of many LIMPpositive accumulations scattered throughout the fibers 24, 25 and the autophagic nature of the vacuoles on electron microscopy.

Moreover, some clusters of autolysosomes are surrounded by membranes with sarcolemmal features but others are not. In support of this notion, ultrastructural studies identified 2 types of autophagic vacuoles: 1 clusters of autophagic vacuoles not surrounded by membranes or basal lamina, and 2 vacuoles containing various autophagic materials encircled by membranes with basal lamina along the luminal side.

In contrast, muscle fibers with LIMPpositive autolysosomal accumulations existed even in young patients and decreased slightly with age. It is most likely that the formation of these autolysosomal accumulations, which are clusters of autophagic vacuoles seen on electron microscopy, is a primary change in muscle fibers of Danon disease and related AVMs.

The formation of peculiar membranes with sarcolemmal features around the autophagic vacuoles is hence a secondary phenomenon.

Since muscle symptoms progress slowly in Danon disease, the development of muscle symptoms might be associated more closely with the formation of the unusual autophagic vacuoles rather than directly with the deficiency of LAMP LAMP-1 is the autosomal paralogous counterpart of LAMP-2 and both are thought to protect lysosomal membrane and cytoplasm from proteolytic enzymes within the lysosomes.

LAMP-2 is tissue-specific but unlike LAMP 1, which is ubiquitously expressed, its expression is likely to be specifically regulated Inhibition of LAMP-1 function results in failure of fusion of lysosomal and plasma membranes and therefore impaired exocytosis 27 , a process usually by which cytoplasmic debris in the autophagosomes are extruded out from the cell through the sarcolemma We therefore assume that LAMP-2 deficiency might likewise be related to dysregulation of exocytosis, leading to the development of the unusual autophagic vacuoles with unique sarcolemmal features.

We revealed the presence of all of these proteins in the fibers with autophagic vacuoles, indicating that in addition to the lysosomal system, the endosomal system is activated in Danon disease and related AVMs.

Interestingly, VAMP-7 was increased in nonvacuolated fibers without autolysosomal accumulations, suggesting that maturation to late endosomes could prevent the formation of the unique vacuolar membranes.

Most of the vacuolar membranes were closed and were not connected to the sarcolemma in Danon disease. The autolysosomes containing cytoplasmic debris are therefore seen to be entrapped within the lumen of the vacuoles, and as such can be possibly considered to be extracellular space.

Together with the observations that most AVSF did not accumulate in the subsarcolemmal region but were scattered in the cytoplasm, our findings suggest that the unique vacuolar membranes may be formed in situ in cytoplasm by a mechanism other than indentation of sarcolemma.

One hypothesis is that the vacuolar membrane with basal lamina might be produced around clusters of autolysosomes Fig.

The membranes surrounding the autophagic vacuoles might have originated from the lysosomal membrane or the isolation membrane that elongates and develops into the membrane of autophagosome 30 , or is formed in situ and entirely de novo.

If the vacuolar membranes are formed within the muscle fibers, it is compatible with the observation that the vacuolar membranes lack collagens IV and VI because collagens are thought to be produced mainly in the interstitium.

Further studies are still necessary to understand the mechanism of the formation of these peculiar vacuolar membranes. Schematic diagram of autophagic vacuoles in muscle fiber of patients with Danon disease.

The membranes of the AVSFs were closed and were not connected to the sarcolemma. We suggest that the unique vacuolar membranes may be formed in situ in cytoplasm by a mechanism.

One hypothesis is that the vacuolar membrane with basal lamina might be produced around clusters of autolysosomes, as illustrated.

However, the strong similarity of the pathologic characteristics to Danon disease naturally raised the question of whether AChE activity is present in the vacuolar membranes in XMEA.

The observation that some features are not seen in Danon disease, like the presence of multilayered basal lamina and the deposition of C5b-9 over the surface of the muscle fiber, raise a possibility that some of these diseases might be allelic to XMEA, albeit different clinical phenotypes.

The autophagic vacuoles in AMD and rimmed vacuoles were reported to occasionally show presence of dystrophin. Nevertheless, these vacuoles are distinct from the AVSF seen in Danon disease and related AVMs, because the frequency of the vacuoles with sarcolemmal features is much less and most of sarcolemmal proteins are not consistently present in the vacuolar membranes of AMD and the rimmed vacuolar myopathies.

However, type 1 vacuoles were thought to be open to extracellular space and arise from invagination of the sarcolemma. Moreover, the membranes of AVSF have not only many sarcolemmal and extracellular proteins but also AChE activity, and may be formed in situ in cytoplasm as described above.

Therefore, we think that the AVSF are a new, highly specific subtype of type 1 vacuoles. Most likely, this unique pathologic finding will probably be found in more diseases and therefore the list of AVMs in this group is likely to expand.

The authors thank Drs. Shuzo M. We also thank Dr. Janice E. The authors thank Ms. Nature ; : — Google Scholar.

Lysosomal glycogen storage disease with normal acid maltase. Neurology ; 31 : 51 — The clinicopathological features of genetically confirmed Danon disease.

Neurology ; 58 : — Danon disease and related disorders. Acta Myologica ; 20 : — Sarcolemmal indentation in cardiomyopathy with mental retardation and vacuolar myopathy.

Neuromuscul Disord ; 5 : — X-linked myopathy with excessive autophagy: A new hereditary muscle disease. Ann Neurol ; 23 : — Infantile autophagic vacuolar myopathy is distinct from Danon disease.

Neurology ; 57 : — 5. A novel form of autophagic vacuolar myopathy with late-onset and multiorgan involvement. Neurology ; 61 : — Nishino I.

Autophagic vacuolar myopathies. Curr Neurol Neurosci Rep ; 3 : 64 — Accumulation of autophagic vacuoles and cardiomyopathy in LAMPdeficient mice. Nature ; : — 6.

Trends Mol Med ; 7 : 37 — Characterization of Danon disease in a male patient and his affected mother. Neuromuscul Disord ; 13 : — Distal myopathy with rimmed vacuoles is allelic to hereditary inclusion body myopathy.

Neurology ; 59 : — Dystrophin-related protein in skeletal muscles in neuromuscular disorders: Immunohistochemical study.

Acta Neuropathol ; 85 : — Dystrobrevin deficiency at the sarcolemma of patients with muscular dystrophy. Hum Mol Genet ; 6 : — Sarcolemmopathy: Muscular dystrophies with cell membrane defects.

Brain Pathol ; 11 : — Primary structure of dystrophin-related protein. J Biol Chem ; : — Mutations in the caveolin-3 gene cause autosomal dominant limb-girdle muscular dystrophy.

Nat Genet ; 18 : — Dysferlin is a surface membrane-associated protein that is absent in Miyoshi myopathy. Neurology ; 53 : — Type VI collagen anchors endothelial basement membranes by interacting with type IV collagen.

Fine structural distribution of acetylcholine receptors at developing mouse neuromuscular junctions. J Neurosci ; 3 : — Ishikawa Y Shimada Y.

Acetylcholine receptors and cholinesterase in developing chick skeletal muscle fibers. Brain Res ; : — Fukuda M.

Biogenesis of the lysosomal membrane. Subcell Biochem ; 22 : — Winchester BG. Lysosomal membrane proteins. Eur J Paediat Neurol ; 5 : 11 — Cell surface expression of lysosome-associated membrane protein-2 lamp2 and CD63 as markers of in vivo platelet activation in malignancy.

Eur J Haematol ; 55 : — Cell ; : — Engel AG. Acid maltase deficiency. Myology, Vol. Google Preview. VAMP-7 mediates vesicular transport from endosomes to lysosomes.

J Cell Biol ; : — Dissection of autophagosome formation using Apg5-deficient mouse embryonic stem cells. Am J Pathol ; : — Laminin-induced acetylcholine receptor clustering: An alternative pathway.

Oxford University Press is a department of the University of Oxford. It furthers the University's objective of excellence in research, scholarship, and education by publishing worldwide.

Sign In or Create an Account. Sign In. Advanced Search. Search Menu. Article Navigation. Close mobile search navigation Article Navigation. Volume Locomotive syndrome in participants with obesity was more frequent than those without obesity, while locomotive syndrome in participants with an exercise habit was less frequent than those without an exercise habit.

Based on 1 article published since Why 1 article? Between and , Eri Arikawa-Hirasawa wrote the following article about Aged.

Copyright C by Expertscape Inc.. A nationwide observational study of locomotive syndrome in Japan using the ResearchKit: The Locomonitor study.

The autolysosomes containing cytoplasmic debris are therefore seen to be entrapped within the lumen of the vacuoles, and as such can be possibly considered to be extracellular space.

Together with the observations that most AVSF did not accumulate in the subsarcolemmal region but were scattered in the cytoplasm, our findings suggest that the unique vacuolar membranes may be formed in situ in cytoplasm by a mechanism other than indentation of sarcolemma.

One hypothesis is that the vacuolar membrane with basal lamina might be produced around clusters of autolysosomes Fig. The membranes surrounding the autophagic vacuoles might have originated from the lysosomal membrane or the isolation membrane that elongates and develops into the membrane of autophagosome 30 , or is formed in situ and entirely de novo.

If the vacuolar membranes are formed within the muscle fibers, it is compatible with the observation that the vacuolar membranes lack collagens IV and VI because collagens are thought to be produced mainly in the interstitium.

Further studies are still necessary to understand the mechanism of the formation of these peculiar vacuolar membranes. Schematic diagram of autophagic vacuoles in muscle fiber of patients with Danon disease.

The membranes of the AVSFs were closed and were not connected to the sarcolemma. We suggest that the unique vacuolar membranes may be formed in situ in cytoplasm by a mechanism.

One hypothesis is that the vacuolar membrane with basal lamina might be produced around clusters of autolysosomes, as illustrated. However, the strong similarity of the pathologic characteristics to Danon disease naturally raised the question of whether AChE activity is present in the vacuolar membranes in XMEA.

The observation that some features are not seen in Danon disease, like the presence of multilayered basal lamina and the deposition of C5b-9 over the surface of the muscle fiber, raise a possibility that some of these diseases might be allelic to XMEA, albeit different clinical phenotypes.

The autophagic vacuoles in AMD and rimmed vacuoles were reported to occasionally show presence of dystrophin.

Nevertheless, these vacuoles are distinct from the AVSF seen in Danon disease and related AVMs, because the frequency of the vacuoles with sarcolemmal features is much less and most of sarcolemmal proteins are not consistently present in the vacuolar membranes of AMD and the rimmed vacuolar myopathies.

However, type 1 vacuoles were thought to be open to extracellular space and arise from invagination of the sarcolemma. Moreover, the membranes of AVSF have not only many sarcolemmal and extracellular proteins but also AChE activity, and may be formed in situ in cytoplasm as described above.

Therefore, we think that the AVSF are a new, highly specific subtype of type 1 vacuoles. Most likely, this unique pathologic finding will probably be found in more diseases and therefore the list of AVMs in this group is likely to expand.

The authors thank Drs. Shuzo M. We also thank Dr. Janice E. The authors thank Ms. Nature ; : — Google Scholar. Lysosomal glycogen storage disease with normal acid maltase.

Neurology ; 31 : 51 — The clinicopathological features of genetically confirmed Danon disease. Neurology ; 58 : — Danon disease and related disorders.

Acta Myologica ; 20 : — Sarcolemmal indentation in cardiomyopathy with mental retardation and vacuolar myopathy. Neuromuscul Disord ; 5 : — X-linked myopathy with excessive autophagy: A new hereditary muscle disease.

Ann Neurol ; 23 : — Infantile autophagic vacuolar myopathy is distinct from Danon disease. Neurology ; 57 : — 5. A novel form of autophagic vacuolar myopathy with late-onset and multiorgan involvement.

Neurology ; 61 : — Nishino I. Autophagic vacuolar myopathies. Curr Neurol Neurosci Rep ; 3 : 64 — Accumulation of autophagic vacuoles and cardiomyopathy in LAMPdeficient mice.

Nature ; : — 6. Trends Mol Med ; 7 : 37 — Characterization of Danon disease in a male patient and his affected mother. Neuromuscul Disord ; 13 : — Distal myopathy with rimmed vacuoles is allelic to hereditary inclusion body myopathy.

Neurology ; 59 : — Dystrophin-related protein in skeletal muscles in neuromuscular disorders: Immunohistochemical study. Acta Neuropathol ; 85 : — Dystrobrevin deficiency at the sarcolemma of patients with muscular dystrophy.

Hum Mol Genet ; 6 : — Sarcolemmopathy: Muscular dystrophies with cell membrane defects. Brain Pathol ; 11 : — Primary structure of dystrophin-related protein.

J Biol Chem ; : — Mutations in the caveolin-3 gene cause autosomal dominant limb-girdle muscular dystrophy. Nat Genet ; 18 : — Dysferlin is a surface membrane-associated protein that is absent in Miyoshi myopathy.

Neurology ; 53 : — Type VI collagen anchors endothelial basement membranes by interacting with type IV collagen.

Fine structural distribution of acetylcholine receptors at developing mouse neuromuscular junctions. J Neurosci ; 3 : — Ishikawa Y Shimada Y.

Acetylcholine receptors and cholinesterase in developing chick skeletal muscle fibers. Brain Res ; : — Fukuda M.

Biogenesis of the lysosomal membrane. Subcell Biochem ; 22 : — Winchester BG. Lysosomal membrane proteins. Eur J Paediat Neurol ; 5 : 11 — Cell surface expression of lysosome-associated membrane protein-2 lamp2 and CD63 as markers of in vivo platelet activation in malignancy.

Eur J Haematol ; 55 : — Cell ; : — Engel AG. Acid maltase deficiency. Myology, Vol. Between and , Eri Arikawa-Hirasawa wrote the following article about Aged.

Copyright C by Expertscape Inc.. A nationwide observational study of locomotive syndrome in Japan using the ResearchKit: The Locomonitor study.

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