Search PubMed⌕ Search

Biomedical subjects

M Elleder

Publications and source records attributed to M Elleder.

At least 145 records · Page 8Linked to original sources
PubMed1981

Histochemical and ultrastructural study of Gaucher cells.

Findings are presented of the histochemical and electron microscopical studies of several bioptic specimens of a juvenile form of Gaucher's disease. The storage was restricted to the macrophages with the exception of occasional hepatocytes in which structures closely resembling the storage tubules were found. The cytoplasmic striations of GC were found to be composed both of lipid and a glycoprotein components which can be easily dissociated from each other by means of either organic solvent extraction or proteolytic digestion. According to the results obtained it is the glycoprotein component which is mainly responsible for the cytoplasmic striations. On the contrary the majority of the tubular storage material of the Krabbe cells was found to be removed with chloroform methanol. Results of the enzyme histochemical study (hydrolases and dehydrogenases) point to the considerably higher overall metabolic activity of GC when compared to the Niemann-Pick storage macrophages.

Gaucher Disease↗
PubMed1981

[Morphogenesis of Gaucher's cells and cells similar to them (in myelosis)].

The cytohistological resemblance between Gaucher's cells and similar cells of the bone marrow in cases of myelosis is great but their electronoptical pictures vary,. In Gaucher's cells there are membranous limited and non-limited corpuscles, composed of typical convoluted tubules, which do not contain lipids, they may be found even in the cisterns of the rough endoplasmatic reticulum and in perinuclear cisterns. They probably represent the proteinic bearer of glucosyl-ceramids, which are extracted during standard histological techniques and therefore do not form part of the histological picture. The gradual loss of the membraneceous lining around these corpuscles is probably the expression of differently advanced states of lysosomatic break down. The Gaucherlike cells of the bone marrow are apparently macrophages with many cytolysosomes, the tubular portions of which are usually not membranous limited, do not have any tendency of screwing and they differ from the previous ones in many other details.

Bone Marrow↗
PubMed1980

[Outline of the pathology of the Langerhans cell system].

About 100 biopsy specimens, most of them from the skin, were used for comparisons between high alpha-D-mannosidase activity and the presence of Langerhans' cells identified according to the electronoptic picture. Agreement between the two was absolute so that alpha-D-mannosidase could be rated as a reliable index of Langerhans' cells not only in histiocytosis X but also in a number of non-tumour and other cancerous processes in the skin. -- Contact couples (apposition) of Langerhans' cells and lymphocytes were often found in as well as outside the epidermis in mycosis fungoides just as in skin allergoes of the contact dermatitis type (less so in experimental alergic dermatitis in the rat, guinea-pig, and rabbit caused by dinitrochlorobenzene). However, specific examinations of a number of electronograms from skin biopsies fairly often revealed such couples also in seborrhoeic keratosis, in basaliomas, and in other processes. -- Despite the above mentioned dispersion variance of the results obtained, Langerhans' cells may yet be found to represent a first link in a chain of immunity responses to exogenous antigens connected with ecotaxis and leading up to a picture of skin allergoses, dermatopathic reticulosis and mycosis fungoides.

Animals↗
PubMed1979

Alkaline Phosphatase Activity Induction in Human Spleen Sinuses in Storage Diseases.

Human splenic sinuses were observed for the induction of alkaline phosphatase (AP) activity in mucopolysaccharidoses of type I and II, in GM1 gangliosidosis, and in Niemann-Pick's disease, type A. A substantially lower degree of activity was found in Sanfillipo's disease, type A, and in hemosiderin pigmentation of the sinuses. In a number of hematological affections and in control spleens AP activity could not be proved by histochemical means. From the formal pathogenetic view, enzyme activity induction is probably related to lysosomal deposition of the material stored.

Enzyme Induction↗
PubMed1979

Comments on spleen sinus enzyme equipment. A histochemical study.

Histochemical observations were made of the activities of nucleosidephosphatases splitting ATP, ADP, IDP, and AMP and exopeptidases splitting l-alanine, l-leucine and l-glycyl-proline in the spleen sinuses of man, mouse, rat, hamster, and rabbit. Of the exopeptidases, only glycylprolyl-naphthylamidase could be proved histochemically, and that only in man and rat. Nucleosidephosphatases showed only traces of activity except in the rabbit where there was highly active AMP-ase, the others being moderately active.

Acid Anhydride Hydrolases↗
PubMed1978

A histochemical and ultrastructural study of stored material in neuronal ceroid lipofuscinosis.

A histochemical and ultrastructural study of five cases of neuronal ceroid lipofuscinosis (NCL) revealed the existence of two related lipopigments differing in some tinctorial properties and ultrastructure. Type I pigment is present in all the tissues affected and corresponds to the pigmentary tertiary lysosomes of well known ultrastructure. Type II pigment occurs exclusively in the neurones of lipophilic cerebral grisea, as a component of the so called protein-myoclonic bodies. It shares with type I certain basic tinctorial properties of lipopigment and its lysosomal localization, but differs in other respects. It stains poorly if at all with the PAS and PAF techniques and is markedly metallophilic, azurophilic and positive for protein. Type II pigment is extremely electron-opaque after staining with heavy metals to the extent that they appear practically amorphous. The possibility that type II material is derived from type I pigment is considered. The amount of type II pigment is highly variable. Both types of pigments are present in residual bodies of various shape and size, including spheroids.

Brain Chemistry↗
PubMed1978

An unusual case of phospholipidosis.

We present the results of a structural, histochemical and lipid-chromatographic study of tissues obtained at postmortem from an unusual case of phospholipidosis. A previous biopsy of the appendix and liver (Elleder et al., 1975a) had revealed a predominance of phosphoglyceride storage, principally of lysobisphosphatidic acid (LBPA) postmortem material showed that this lipid was stored exclusively in central neurons. In the spleen and the lymph node, however, sphingomyelin (SP) was shown, histochemically and chromatographically, to be the main lipid stored. Total sphingomyelinase (SPase) activity in the appendix was reduced to about 50% of normal. Neuroaxonal dystrophy (NAD) and a conspicuous discrepancy between the degree of distension of some neurons and their lipid content deserve special mention. The case is contrasted with classical sphingomyelinosis; the complexity of the Niemann-Pick group of diseases is discussed as an indication of the difficulties of classification of any atypical case.

Appendix↗
PubMed1978

[Histochemistry as a diagnostic technic in the classification of hematopoietic-system tumors].

106 tumours involving the haematopoietic system were examined using a wider range of histochemical methods, particularly enzymatic ones. The results were compared with other diagnostic techniques. A critical analysis of the results showed that as regards the purely diagnostic aspect the significance of histochemistry was severely limited to practically no more than tumours of the granulocytic series (myeloperoxidase and chloracetylesterase activities). Proof of alpha--mannosidase activity appeared to be greatly promising in the group of histiocytosis X. In the rest of the series, particularly in the lymphatic and thrombocytic series, the diagnostic significance of histochemistry proved to be either limited or non-existent. Considering the present state of methods used, the main advantage of histochemistry is in that it helps add details to the metabolic profile of the tumour under study. Some of the problems of the conception of the so called markers are discussed.

Hematopoietic System↗
PubMed1978

[Histological, electron-optical and histochemical findings in mycosis fungoides].

Skin excisions were investigated in 5 patients with verified mycosis fungoides. Findings yielded by light and electron microscopy helped to confirm the findings anticipated, and were correlated with histochemical observations. One of the cases involved a substantially higher activity of lysosomal enzymes, particularly KF and beta-glucuronidase in the skin infiltrate mycotic cells. In the other cases, this sort of activity was low. The significance of high ATPase activity in the peripheral cell membrane remains unclear. In one case, involvement in the T lymphocyte series was confirmed by the formation of rosettes.

Aged↗
PubMed1977

Lysosomal non-lipid component of Gaucher's cells.

An ultrastructural, histochemical and chemical analysis of storage elements in the infantile form of Gaucher's disease showed that in addition to cerebroside the lysosomes also included a non-lipid component of protein, or possibly glycoprotein nature. This component, easily removable with trypsin, was present in such quantities that it conditioned the typical solid and fibrillar appearance of storage elements even after they had been substantially delipidized. Another noteworthy finding was that the ultrastructural appearance of tubular structures generally regarded as stored cerebrosides persisted in all the extracted specimens without any noticeable change. The findings are compared with available data from the literature and their significance briefly discussed.

Cerebrosides↗