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R. D. Martinus P. A. W. Harper R. D. Jolly S. L. Bayliss G. G. Midwinter G. J. Shaw D. N. Palmer 《Veterinary research communications》1991,15(2):85-94
The ceroid-lipofuscinoses (Batten's disease) are a group of recessively inherited lysosomal storage diseases of children and animals in which there is intracellular accumulation of a fluorescent lipopigment in a wide variety of cells. Lipopigment bodies isolated from pancreas, liver, kidney and brain tissue from a heifer affected with ceroid-lipofuscinosis contained between 55 and 62% protein. A dominant component comigrated on LDS-PAGE with the major low molecular weight protein stored in ovine ceroid-lipofuscinosis. It was identified by amino acid sequence and mass spectroscopy as the full subunit c of mitochondrial ATP synthase, normally found only in the inner mitochondrial membrane, where it is estimated to account for 2–4% of the membrane protein. In pancreatic lipopigment it accounted for at least 40% of the total lipopigment mass and this storage was considered specific to the disease. No other mitochondrial proteins were found in storage bodies. These results are similar to those found in studies on the ovine and the late infantile and juvenile human forms of the disease. It is concluded that bovine ceroid-lipofuscinosis is also a proteolipid proteinosis in which subunit c of mitochondrial ATP synthase is specifically stored in lysosome derived organelles.Abbrevations DCCD
dicyclohexycarbodi-imide
- LDS-PAGE
lithium dodecyl sulphate polyacryl-amide gel electrophoresis
- PTH
phenylthiohydantoin
- CsCl
caesium chloride 相似文献
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Grete M. Kindberg Birgit H. Dannevig Knut-Jan Andersen Trond Berg 《Fish physiology and biochemistry》1991,9(2):113-121
The intracellular handing of a mannose-terminated glycoprotein taken up in rainbow trout liver cells by receptor-mediated endocytosis has been studied. The intracellular transport and degradation of ovalbumin (OA) were studied by means of subcellular fractionation in Nycodenz gradients and by differential centrifugation following intravenous injection of the ligand. By using OA labelled with125I-tyramine cellobiose (125I-TC), the subcellular distribution of labelled degradation products could be studied, since they are trapped intracellularly in the organelle where the degradation takes place.
125I-TC-OA was shortly after injection (45 min) localized in a homogenous population of endosomes. Labelled degradation products firs appeared in an organelle with the same density distribution as the endosomes. In livers homogenized 2h after injection the degradation products appeared in organelles with increasing size and density. After 24h, the degradation products were recovered in at least two populations of lysosomes with a distribution profile which coincided with that of the lysosomal enzyme -acetylglucosaminidase.The heterogeneous distribution of the late degradation products seemed not to be due to uptake of ligand in different liver cell types as only the parenchymal liver cells took up labelled OA after intravenous injection of the ligand. 相似文献
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A Ryter 《Comparative immunology, microbiology and infectious diseases》1985,8(2):119-133
The main function of the macrophages, which is to ingest and degrade any foreign molecules or particles penetrating the organism, appears in the development of the different structures implicated in endocytic activity. The macrophage's high endocytic property first appears in its irregular shape and the large number of extensions of the cell membrane, allowing the rapid capture of extra-cellular material. Adhesion between macrophage cell surface and molecules or particles is greatly enhanced by the presence of varied kinds of receptors: lectin-like receptors which bind specific sugars or highly specific receptors such as Fc and C3b receptors, which increase phagocytosis of opsonized microbes. The microbicidal properties reside in part in the production of superoxide anions which result from the activity of a NAD(P)H oxidase. This enzyme is located in the plasma membrane. Its activity could be demonstrated with a cytochemical method, on the cell surface and along the phagosome membrane. It is, however, very weak in resident macrophages and increases after stimulation or activation. The second kind of bactericidal property corresponds to cationic proteins located in lysosomes. After fusion between lysosomes and phagosomes, they contribute to microbe killing by permeabilizing microbe envelopes. Lysosomes, which contain diverse acid hydrolases and are responsible for the degradation of ingested material, play a crucial role in macrophage endocytic activity. Their number increases in parallel with endocytic activity during macrophage differentiation and is particularly high after ingestion of degradable material. Contrary to polymorphonuclear leukocytes, macrophage is very poor in granules containing peroxidase. The latter, which are rather abundant in monocytes, disappear during macrophage maturation. They do not seem thus to be implicated in macrophage microbicidal activity. Endocytosis is accompanied by rapid and intense exchanges between the different membrane compartments of the cell (plasma membrane, pinosomes or phagosomes, endosomes, lysosomes, Golgi apparatus, etc.). These exchanges seem to occur by transitory fusions between vesicles coming from different compartments, rapidly followed by their recycling to their original compartment. This system of membrane shuttle has been clearly observed after formation of phagosomes or pinosomes in which the internalized plasma membrane is recycled back to the cell surface within a few minutes after their formation. This membrane traffic is especially intense in macrophages, the endocytic activity of which is very high, but it also exists in all cell types.(ABSTRACT TRUNCATED AT 400 WORDS) 相似文献
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Thorsten Mordhorst Sushil Awal Sebastian Jordan Charlotte Petters Linda Sartoris Ralf Dringen Ulf Bickmeyer 《Marine drugs》2015,13(2):920-935
Based on the chemical structure and the known chemical synthesis of the marine sponge alkaloid ageladine A, we synthesized the ageladine A-derivative 4-(naphthalene-2-yl)-1H-imidazo[4,5-c]pyridine trifluoroacetate (LysoGlow84). The two-step synthesis started with the Pictet-Spengler reaction of histamine and naphthalene-2-carbaldehyde to a tetrahydropyridine intermediate, which was dehydrogenated with activated manganese (IV) oxide to LysoGlow84. Structure and purity of the synthesized LysoGlow84 were confirmed by NMR spectroscopy and mass spectrometry. The fluorescence intensity emitted by LysoGlow84 depended strongly on the pH of the solvent with highest fluorescence intensity recorded at pH 4. The fluorescence maximum (at 315 nm excitation) was observed at 440 nm. Biocompatibility of LysoGlow84 was investigated using cultured rat brain astrocytes and the marine flatworm Macrostomum lignano. Exposure of the astrocytes for up to 6 h to micromolar concentrations of LysoGlow84 did not compromise cell viability, as demonstrated by several viability assays, but revealed a promising property of this compound for staining of cellular vesicles. Conventional fluorescence microscopy as well as confocal scanning microscopy of LysoGlow84-treated astrocytes revealed co-localization of LysoGlow84 fluorescence with that of LysoTracker® Red DND-99. LysoGlow84 stained unclear structures in Macrostomum lignano, which were identified as lysosomes by co-staining with LysoTracker. Strong fluorescence staining by LysoGlow84 was further observed around the worms’ anterior gut and the female genital pore which were not counterstained by LysoTracker Red. Thus, LysoGlow84 is a new promising dye that stains lysosomes and other acidic compartments in cultured cells and in worms. 相似文献
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