Biomaterial Database

Copper accumulation in primary biliary cirrhosis. An electron and X-ray microanalytical study. 1982

W Humbert, and M Aprahamian, and C Stock, and J F Grenier

Ultrastructural and X-Ray microanalytical aspects of primary biliary cirrhosis were analysed and compared to those of normal liver. Electron microscopic studies showed a significant increase of dense lipofuscin-like granules in PBC compared to the hepatocytes of normal liver (665%). These granules (0.3-2.7 micrometers in diameter) were formed by the association of primary lysosomes and lipid droplets. X-Ray microanalysis of these structures showed the presence of Calcium, Phosphorus, Potassium, Chlorine, Sulphur, Aluminium and Copper. The coexistence of Sulphur with other mineral elements suggests the occurrence of metalloproteins binding these elements. These findings provide evidence that intralysosomal sequestration of mineral elements, especially copper protects the hepatocytes from cytotoxic effects.

UI	MeSH Term	Description	Entries
D008062	Lipofuscin	A naturally occurring lipid pigment with histochemical characteristics similar to ceroid. It accumulates in various normal tissues and apparently increases in quantity with age.
D008099	Liver	A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.	Livers
D008105	Liver Cirrhosis, Biliary	FIBROSIS of the hepatic parenchyma due to obstruction of BILE flow (CHOLESTASIS) in the intrahepatic or extrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC; BILE DUCTS, EXTRAHEPATIC). Primary biliary cholangitis involves the destruction of small intra-hepatic bile ducts and decreased bile secretion. Secondary biliary cholangitis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes.	Biliary Cirrhosis,Biliary Cirrhosis, Primary,Biliary Cirrhosis, Secondary,Cholangitis, Chronic Nonsuppurative Destructive,Liver Cirrhosis, Obstructive,Primary Biliary Cholangitis,Biliary Cirrhosis, Primary, 1,Primary Biliary Cirrhosis,Secondary Biliary Cholangitis,Secondary Biliary Cirrhosis,Biliary Cholangitides, Primary,Biliary Cholangitis, Primary,Biliary Cholangitis, Secondary,Cholangitides, Primary Biliary,Cholangitis, Primary Biliary,Cholangitis, Secondary Biliary,Cirrhosis, Biliary,Cirrhosis, Secondary Biliary,Liver Cirrhoses, Biliary,Obstructive Liver Cirrhosis,Primary Biliary Cholangitides,Secondary Biliary Cholangitides
D008247	Lysosomes	A class of morphologically heterogeneous cytoplasmic particles in animal and plant tissues characterized by their content of hydrolytic enzymes and the structure-linked latency of these enzymes. The intracellular functions of lysosomes depend on their lytic potential. The single unit membrane of the lysosome acts as a barrier between the enzymes enclosed in the lysosome and the external substrate. The activity of the enzymes contained in lysosomes is limited or nil unless the vesicle in which they are enclosed is ruptured or undergoes MEMBRANE FUSION. (From Rieger et al., Glossary of Genetics: Classical and Molecular, 5th ed).	Autolysosome,Autolysosomes,Lysosome
D008854	Microscopy, Electron	Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen.	Electron Microscopy
D008903	Minerals	Native, inorganic or fossilized organic substances having a definite chemical composition and formed by inorganic reactions. They may occur as individual crystals or may be disseminated in some other mineral or rock. (Grant & Hackh's Chemical Dictionary, 5th ed; McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)	Mineral
D003300	Copper	A heavy metal trace element with the atomic symbol Cu, atomic number 29, and atomic weight 63.55.	Copper-63,Copper 63
D004577	Electron Probe Microanalysis	Identification and measurement of ELEMENTS and their location based on the fact that X-RAYS emitted by an element excited by an electron beam have a wavelength characteristic of that element and an intensity related to its concentration. It is performed with an electron microscope fitted with an x-ray spectrometer, in scanning or transmission mode.	Microscopy, Electron, X-Ray Microanalysis,Spectrometry, X-Ray Emission, Electron Microscopic,Spectrometry, X-Ray Emission, Electron Probe,X-Ray Emission Spectrometry, Electron Microscopic,X-Ray Emission Spectrometry, Electron Probe,X-Ray Microanalysis, Electron Microscopic,X-Ray Microanalysis, Electron Probe,Microanalysis, Electron Probe,Spectrometry, X Ray Emission, Electron Microscopic,Spectrometry, X Ray Emission, Electron Probe,X Ray Emission Spectrometry, Electron Microscopic,X Ray Emission Spectrometry, Electron Probe,X-Ray Microanalysis,Electron Probe Microanalyses,Microanalyses, Electron Probe,Microanalysis, X-Ray,Probe Microanalyses, Electron,Probe Microanalysis, Electron,X Ray Microanalysis,X Ray Microanalysis, Electron Microscopic,X Ray Microanalysis, Electron Probe
D006651	Histocytochemistry	Study of intracellular distribution of chemicals, reaction sites, enzymes, etc., by means of staining reactions, radioactive isotope uptake, selective metal distribution in electron microscopy, or other methods.	Cytochemistry
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man