Biomaterial Database

Glycoconjugates in storage cytosomes from ceroid-lipofuscinosis (Batten's disease) and in lipofuscin from old-age brain. 1989

N A Hall, and B D Lake, and D N Palmer, and R D Jolly, and A D Patrick

Department of Clinical Biochemistry, Institute of Child Health, London, England.

The ceroid-lipofuscinoses (CL) are a group of inherited diseases characterised by the accumulation, in brain, of autofluorescent storage cytosomes which have similar histochemical staining properties to lipofuscin, the neuronal wear and tear pigment of old-age brain. The storage cytosomes stain strongly with periodic acid-Schiff reagent (PAS), indicating the presence of carbohydrate. In brain from each childhood form of CL, concentrations of phosphorylated dolichol (Dol-P) are 10- to 20- fold higher than in age-matched controls. Brain Dol-P concentrations are also increased between 2 and 5- fold in several different lipidoses and in elderly subjects. Much of the Dol-P which accumulates is located within the storage cytosomes. Dol-P constitutes 2-3% of the dry weight of storage cytosomes from juvenile and late-infantile CL, and 0.3-0.7% of storage cytosomes from infantile CL, ovine CL and of lipofuscin isolated from old age brain. The bulk of the Dol-P in CL brain and in isolated storage cytosomes is present as dolichyl pyrophosphoryl oligosaccharides (Dol-PP-OS). The constitutions of the oligosaccharide moieties differ in the various forms of the disease. Histochemical analysis of frozen sections of unfixed brain after extraction by various lipid solvents indicates that the major part of the PAS positive intraneuronal material in CL brain and in old-age brain has the extraction properties of Dol-PP-OS. Carbohydrate represents 4-7% of the dry weight of CL storage cytosomes and of lipofuscin. The major monosaccharide components are mannose, N-acetyl glucosamine, glucose and galactose. Depending on the form of the disease studied, up to 40% of this material can be accounted for by Dol-PP-OS. Polyacrylamide gel electrophoresis of storage cytosomes followed by lectin blotting demonstrates several low molecular weight components which bind concanavalin A. These do not coelute with the major protein components and may well be Dol-PP-OS. We conclude that Dol-PP-OS are concentrated in storage cytosomes in CL and are one of their major glycoconjugate components.

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.
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
D009005	Monosaccharides	Single chain carbohydrates that are the most basic units of CARBOHYDRATES. They are typically colorless crystalline substances with a sweet taste and have the same general formula CnH2nOn.	Monosaccharide,Simple Sugar,Simple Sugars,Sugar, Simple,Sugars, Simple
D009472	Neuronal Ceroid-Lipofuscinoses	A group of severe neurodegenerative diseases characterized by intracellular accumulation of autofluorescent wax-like lipid materials (CEROID; LIPOFUSCIN) in neurons. There are several subtypes based on mutations of the various genes, time of disease onset, and severity of the neurological defects such as progressive DEMENTIA; SEIZURES; and visual failure.	Batten Disease,Ceroid Lipofuscinosis, Neuronal, 4B, Autosomal Dominant,Ceroid-Lipofuscinosis, Neuronal,Jansky-Bielschowsky Disease,Kufs Disease,Santavuori-Haltia Disease,Spielmeyer-Vogt Disease,Adult Neuronal Ceroid Lipofuscinosis,Amaurotic Idiocy, Adult Type,Batten-Mayou Disease,Batten-Spielmeyer-Vogt Disease,CLN3-Related Neuronal Ceroid-Lipofuscinosis,CLN4A,CLN4B,Ceroid Lipofuscinosis, Neuronal 3, Juvenile,Ceroid Lipofuscinosis, Neuronal 4,Ceroid Lipofuscinosis, Neuronal, 3,Ceroid Lipofuscinosis, Neuronal, 4A, Autosomal Recessive,Ceroid Lipofuscinosis, Neuronal, Parry Type,Ceroid Storage Disease,Infantile Neuronal Ceroid Lipofuscinosis,Juvenile Batten Disease,Juvenile Cerebroretinal Degeneration,Juvenile Neuronal Ceroid Lipofuscinosis,Kuf's Disease,Kufs Disease Autosomal Recessive,Kufs Disease, Autosomal Dominant,Kufs Disease, Autosomal Recessive,Kufs Type Neuronal Ceroid Lipofuscinosis,Late-Infantile Neuronal Ceroid Lipofuscinosis,Lipofuscin Storage Disease,Lipofuscinosis, Neuronal Ceroid,Neuronal Ceroid Lipofuscinosis,Neuronal Ceroid Lipofuscinosis Juvenile Type,Neuronal Ceroid Lipofuscinosis, Adult,Neuronal Ceroid Lipofuscinosis, Adult Type,Neuronal Ceroid Lipofuscinosis, Infantile,Neuronal Ceroid Lipofuscinosis, Juvenile,Neuronal Ceroid Lipofuscinosis, Late Infantile,Neuronal Ceroid Lipofuscinosis, Late-Infantile,Neuronal Ceroid-Lipofuscinosis,Spielmeyer-Sjogren Disease,Vogt Spielmeyer Disease,Vogt-Spielmeyer Disease,Batten Disease, Juvenile,Batten Diseases, Juvenile,Batten Mayou Disease,Batten Spielmeyer Vogt Disease,CLN3 Related Neuronal Ceroid Lipofuscinosis,CLN3-Related Neuronal Ceroid-Lipofuscinoses,CLN4As,Cerebroretinal Degeneration, Juvenile,Cerebroretinal Degenerations, Juvenile,Ceroid Lipofuscinosis, Neuronal,Ceroid Storage Diseases,Ceroid-Lipofuscinosis, CLN3-Related Neuronal,Disease, Ceroid Storage,Disease, Juvenile Batten,Disease, Kuf's,Disease, Lipofuscin Storage,Disease, Spielmeyer-Sjogren,Disease, Vogt Spielmeyer,Disease, Vogt-Spielmeyer,Jansky Bielschowsky Disease,Juvenile Batten Diseases,Juvenile Cerebroretinal Degenerations,Kuf Disease,Lipofuscin Storage Diseases,Neuronal Ceroid Lipofuscinoses,Neuronal Ceroid-Lipofuscinoses, CLN3-Related,Neuronal Ceroid-Lipofuscinosis, CLN3-Related,Santavuori Haltia Disease,Spielmeyer Disease, Vogt,Spielmeyer Sjogren Disease,Spielmeyer Vogt Disease,Storage Disease, Ceroid,Storage Disease, Lipofuscin
D011104	Polyisoprenyl Phosphate Oligosaccharides	These compounds function as activated glycosyl carriers in the biosynthesis of glycoproteins and glycophospholipids. Include the pyrophosphates.	Isoprenoid Phosphate Oligosaccharides,Oligosaccharides, Isoprenoid Phosphate,Oligosaccharides, Polyisoprenyl Phosphate,Phosphate Oligosaccharides, Isoprenoid,Phosphate Oligosaccharides, Polyisoprenyl
D011485	Protein Binding	The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.	Plasma Protein Binding Capacity,Binding, Protein
D001923	Brain Chemistry	Changes in the amounts of various chemicals (neurotransmitters, receptors, enzymes, and other metabolites) specific to the area of the central nervous system contained within the head. These are monitored over time, during sensory stimulation, or under different disease states.	Chemistry, Brain,Brain Chemistries,Chemistries, Brain
D003208	Concanavalin A	A MANNOSE/GLUCOSE binding lectin isolated from the jack bean (Canavalia ensiformis). It is a potent mitogen used to stimulate cell proliferation in lymphocytes, primarily T-lymphocyte, cultures.
D004288	Dolichol Phosphates	Phosphoric acid esters of dolichol.	Dolichol Monophosphates,Monophosphates, Dolichol,Phosphates, Dolichol
D004591	Electrophoresis, Polyacrylamide Gel	Electrophoresis in which a polyacrylamide gel is used as the diffusion medium.	Polyacrylamide Gel Electrophoresis,SDS-PAGE,Sodium Dodecyl Sulfate-PAGE,Gel Electrophoresis, Polyacrylamide,SDS PAGE,Sodium Dodecyl Sulfate PAGE,Sodium Dodecyl Sulfate-PAGEs