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Hauptseite Themenportale Zufälliger Artikel. Comedy , Slice of Life , Musik. FSK 0 [1]. Naoko Yamada. The results are summarized in Table 1. Longitudinal section shows that the vacuoles are spherical or oval.

By immunohistochemistry, the AVSF reacted for all the tested sarcolemmal and extracellular matrix proteins in the vacuolar membranes in muscle from patients with Danon disease and related AVMs, although reactivity levels of the proteins were variable Table 1 ; Fig.

However, only collagens IV and VI showed less intense reactivity in the vacuolar membranes than that in the sarcolemma.

Most of the AVSFs were scattered throughout the cytoplasm rather than clustered in the subsarcolemmal region. Longitudinal sections demonstrated the oval shape of the AVSF, confirming the closed structure of the vacuoles Fig.

Vacuolar membranes connected to the sarcolemma were seen in only 2 patients; both were more than 20 years old. These autolysosomal accumulations were surrounded by dystrophin-positive membranes in some fibers but not in others Fig.

Muscle fibers with dystrophin-positive vacuoles accounted for 0. Muscle fibers with autolysosomal accumulations, both with and without dystrophin-positive vacuolar membranes, accounted for Indirect immunohistochemistry.

A, B Danon disease patient; C : Normal control. In Danon disease some muscle fibers express both LIMP-1 and dystrophin arrowheads , whereas some muscle fibers show overexpression of LIMP-1 with absence of dystrophin arrows.

Double immunohistochemistry. Transverse sections of skeletal muscle from Danon disease patient, stained for dystrophin and LIMP In some muscle fibers, LIMPpositive accumulations are clearly surrounded by dystrophin immunopositive membrane D-F.

These vacuoles are the AVSF. Relationship between age of the patients with Danon disease and number of muscle fibers with vacuoles highlighted with dystrophin or LIMP-1 on immunohistochemistry.

The open circles show the only patient who had 2 muscle biopsies. Cathepsin L was expressed weakly, mainly in fibers with autolysosomal accumulations.

Only VAMP-7 was strongly expressed, mainly in the nonvacuolated fibers without autolysosomal accumulations.

There were occasional intracytoplasmic vacuoles with sarcolemmal proteins in muscles from patients with other AVMs i.

In AMD, sarcolemmal and extracellular matrix proteins were present in some vacuolar membranes. In AMD, dystrophin is present on some vacuolar materials.

In Danon disease and related AVMs, electron microscopy revealed scattered clusters of autophagic vacuoles containing cytoplasmic debris, electron dense materials, and myeloid bodies.

Some of these autophagic vacuoles had basal lamina on the luminal side, while other clusters were not limited by a membrane Fig. Electron micrograph in muscle from Danon disease patient.

Scattered in the muscle fibers were clusters of autophagic vacuoles A containing cytoplasmic debris, electron dense material, and myeloid bodies.

Some of these clusters were encircled by a membrane with basal lamina paired arrowheads on its luminal side, while other clusters are not limited by a membrane B.

Electron immunohistochemistry after single labeling with dystrophin or LIMP-1 antibody shows localization of the proteins in autophagic vacuoles C-E.

In the clusters with membranes, that is, the AVSF A , the immunogold particles show dystrophin C along the vacuolar membrane paired arrowheads , and the immunogold particles show LIMP-1 D around autophagic material inside autophagic vacuoles.

In contrast, in the clusters not surrounded by membranes, immunogold particles show LIMP-1 around autophagic materials with absence of dystrophin E.

Immunoelectron microscopy showed many autophagic vacuoles; however technical limitations posed by preparing samples from frozen tissue without prefixation prevented us from clearly defining vacuolar membranes.

At higher magnification, dystrophin signals were detected on the cytoplasmic side of the vacuolar membrane and along the periphery of the vacuoles Fig.

In contrast, the LIMP-1 antibody signals were associated with autophagic materials including glycogen particles and cytoplasmic debris within the vacuoles, suggesting that the vacuoles are limited by membranes with sarcolemmal features and contain multiple small autophagic vacuoles derived from autolysosomes.

The granules contained acid phosphatase-positive material. On immunohistochemistry, the vacuolar membranes were stained with antibodies against dystrophin and other sarcolemmal proteins as well as extracellular matrix proteins, whereas LAMP-2 was completely absent in the muscle.

On electron microscopy, there were scattered intracytoplasmic autophagic vacuoles with glycogen particles and cytoplasmic debris data not shown.

In muscle from patients with Danon disease and related AVMs, the membranes of AVSF showed immunoreactivity for all of the sarcolemmal and extracellular matrix proteins tested.

Dystrophin and dystrobrevin are cytoskeletal proteins localized along the cytoplasmic side of the sarcolemma 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.

In subjects without locomotive syndrome 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?

Trends Mol Med ; 7 : 37 — Anime News Network, Shae summers Anime News NetworkTeen lingerie porn. For double immunolabeling using mouse monoclonal anti-LIMP-1 and rabbit polyclonal anti-dystrophin antibodies a generous gift from Dr. Schematic diagram of autophagic vacuoles in muscle fiber of Big boobs red head with Danon disease. The granules contained acid phosphatase-positive material.

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