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Biomedical subjects

A Prelle

Publications and source records attributed to A Prelle.

67 records · Page 4Linked to original sources
PubMed1990

Kearns-Sayre syndrome: different amounts of deleted mitochondrial DNA are present in several autoptic tissues.

A population of deleted mitochondrial DNA (mtDNA) was found in different amounts in autoptic muscle, heart, cortex, cerebellum, liver and kidney of a patient who died of Kearn-Sayre Syndrome (KSS). The widespread occurrence of the deletion correlates with the multisystem nature of KSS and supports the hypothesis that this is a genetic disease due to an alteration of mtDNA presumably arising in the oocyte or early embryo.

Brain↗
PubMed1989

Ultrastructural localization of calcium binding sites on human muscle cell surface.

Calcium (Ca2+) is mainly bound to anionic phospholipids and to sialic acid at the cell surface. We studied the ultrastructural localization of these Ca2+ binding sites in normal human muscle fibers, using Polymyxin B as a marker for anionic phospholipids and the lectin Limulus Polyphemus as a probe for sialic acid. We found that anionic phospholipids have a patchy distribution along the muscle sarcolemma, with a preferential localization at the I band level and at the junction between the I and A band. Sialic acid has an uniform distribution along the muscle plasma membrane and basal lamina. Our observations suggest that the plasma membrane, basal lamina, and transverse tubular system play an important role in providing the negative charge of the human muscle cell surface and that these structures may be involved in the binding of calcium.

Arthropod Proteins↗
PubMed1989

Cytochrome c oxidase during human fetal development.

Histochemical, biochemical and immunologic analysis of cytochrome c oxidase (COX) in skeletal muscle, heart and kidney during human fetal development was performed. COX histochemical activity was present only in few muscle fibres from the 11th to the 20th week of gestation. At the same developmental stage intrafusal muscle fibres, heart and kidney already showed strong activity. At the 28th week of gestation muscular COX activity was present in about 90% of the fibres. Tissue biochemical analysis confirmed these histochemical findings. Histochemical and biochemical findings compared to the immunocytochemical results and ELISA indicate that COX activity parallels the progressive synthesis of the enzyme in each tissue.

Electron Transport Complex IV↗
PubMed1989

Characterization of SV40-transformed human cells by immunofluorescence and fluorescent in situ hybridization techniques.

We have performed immunofluorescent and fluorescent in situ hybridization studies in order to better clarify the integration of SV40 DNA in human fibroblast cell lines. Most of the cells were T-antigen positive by immunocytochemical studies, while in all the cells we detected the integrated viral DNA by in situ hybridization. Both techniques are easy and useful to perform but the molecular genetic method gives a more specific signal with the possibility of localizing molecular hybrids in the nucleus and in the cytoplasm of the transformed cells.

Antigens, Polyomavirus Transforming↗
PubMed1989

Nile red simultaneous staining of intracellular lipids and membrane network in human muscle cultures.

We have applied a new fluorescent probe, Nile red, on normal and pathological human muscle derived cultures and compared the results with corresponding human muscle sections. In normal human muscle cultures, Nile red strain has proved useful for visualization of both intracellular lipids and membrane network. Similar patterns have been observed in muscle cultures derived from lipid storage and mitochondrial myopathies. Moreover, abnormalities in pathological muscle cultures could be revealed by establishing more advanced culture systems.

Cells, Cultured↗
PubMed1988

Immunocytochemical study of nebulin in Duchenne muscular dystrophy.

We used antibodies to nebulin in immunocytochemical studies. In normal muscle, nebulin was localized at the I band. The protein was also present in most fibers from all 15 Duchenne muscular dystrophy (DMD) patients studied, including patients who seemed to lack nebulin in electrophoretic gels and patients who demonstrated deletions of DNA in the region of Xp21. These results conform to other evidence that nebulin is not the primarily affected gene product in DMD; it may be affected secondarily.

Child↗
PubMed1987

Progressive cytochrome c oxidase deficiency in a case of Kearns-Sayre syndrome: morphological, immunological, and biochemical studies in muscle biopsies and autopsy tissues.

We report biochemical, immunological, and morphological findings in a patient with fatal Kearns-Sayre syndrome. Histochemical and biochemical findings from muscle biopsy specimens obtained 7 years apart documented the disease's evolution from a mild mitochondrial disorder affecting a small proportion of muscle fibers to a severe disorder affecting a large proportion of muscle fibers. Cytochrome c oxidase activity in muscle declined profoundly as the disease progressed, although the level of enzyme protein was normal, as shown by immunochemical techniques. Other organs were severely affected by the disease. Examination of postmortem tissue showed spongiosis in the frontal cortex, diffuse loss of Purkinje cells in the cerebellum, liver steatosis, and heart fibrosis with mitochondrial abnormalities. Cytochrome c oxidase activity was only slightly reduced in these organs.

Adult↗
PubMed1987

Application of nile blue and nile red, two fluorescent probes, for detection of lipid droplets in human skeletal muscle.

Using frozen sections from human muscle biopsies, we assessed the value of Nile blue and Nile red, two fluorescent probes, as stains for lipid droplets in normal and pathological skeletal muscle fibers. In normal muscle, lipid storage disorders, and mitochondrial myopathies, Nile blue stained the lipid droplets as yellow-gold fluorescent structures. The lipid droplets were also seen as yellow-gold fluorescent structures in Nile red-stained sections, but the outstanding feature in these preparations was the staining of the membrane network of the muscle fibers and membrane proliferations in pathological muscle as red-orange fluorescent structures. These results suggest that both Nile blue and Nile red stains are useful for visualization of lipid droplets and membrane proliferations in pathological muscle biopsies.

Cell Membrane↗
PubMedDate not supplied

A sporadic, atypical case of desminopathy: morphological and immunological characterization.

Recently, abnormal expression of cyclin-dependent kinases was proposed as a possible cause of desminopathy. We describe an atypical case clinically characterized by severe respiratory distress. Muscle biopsy showed subsarcolemmal and intracytoplasmic accumulation areas, which intensively stained with anti-desmin antibodies and contained electrondense filamentous material at ultrastructural level. WB analysis showed 30% increased desmin signal compared to controls. Positive immunostain for CDC2 kinase, CDK2 and emerin and nuclear matrix-associated protein were, found in desmin-positive fibres.

Adult↗
PubMedDate not supplied

Critically ill patients: immunological evidence of inflammation in muscle biopsy.

AIM AND METHOD: To verify whether muscle necrosis in critically ill patients could be due to an inflammatory process, we tested muscle biopsies from five intensive care patients with different inflammation-specific immunocytochemical markers (antibodies anti-class I major histocompatibility complex products (class I MHCP or HLA I), membrane attack complex (MAC), T lymphocytes helper-inducer (CD4), cytotoxic (CD8) and pan-B-lymphocytes). RESULTS: In three patients muscle biopsy showed class I MHCP positivity on the surface membrane of several groups of fibres, mainly perifascicular, and scattered microvascular deposits of MAC. In the other two patients muscle biopsy did not show class I MHCP and MAC positivity. CONCLUSION: Our results suggest that inflammation may be a component of muscle damage in some critically ill patients.

Adult↗