Glycoconjugates in Storage Cytosomes from Ceroid-Lipofuscinosis (Batten’s Disease) and in Lipofuscin from Old-Age Brain

Na, Hall; Lake, B D; Dn, Palmer; Jolly, RD; Ad, Patrick

doi:10.1007/978-1-4899-5339-1_16

Cited by 11 publications

(7 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In the case of human lipofuscin, for example, it has been found that it reacted with concanavalin A (specific for O~-Dmannosyl and glucosyl residues) (Elleder 1989;Wisniewski and Maslinska 1990) but not with other lectins specific for O~-D-acetylgalactosamine, [3-acetylgalactosamine, ~3-D-galactose, and o~-L-fucose (Wisniewski and Maslinska 1990). These findings appear in clear contrast to the biochemical results obtained in lipofuscin granules isolated from the brain of old human subjects indicating the presence of galactose, mannose, glucose, acetylated glucosamine, galactosamine and sialic acid (Hooghwinkel et al 1986;Hall et al 1989).…”

Section: Introductioncontrasting

confidence: 85%

Lectin histochemistry of lipofuscin and certain ceroid pigments

Monserrat

Benavides

Berra

et al. 1995

Histochem Cell Biol

View full text Add to dashboard Cite

Little is known at present about the saccharide components of lipofuscin (age pigment) and ceroid pigments in situ. The purpose of this study was, therefore, to study in detail the lectin reactivities of lipofuscin in neurons and cardiac myocytes of old humans and rats. In addition, those of diverse ceroid pigments found in human aortic atheromas, in the livers of choline-deficient rats, in the uteri of vitamin E-deficient rats and in the crushed epididymal fat pad of rats, are included. Cryostat and deparaffinized sections from all these tissues were either extracted with a solvent mixture of chloroform-methanol-water (10:10:3, v/v) and incubated with 7 different biotinylated lectins or left untreated. Delipidation was done in order to study whether it was possible to discriminate between the saccharide moieties of glycolipids and glycoproteins of lipofuscin and ceroid pigments in situ. Other similarly treated sections were used to study the autofluorescence, sudanophilia, acid-fastness and reactivity to PAS. The frequency and intensity of lectin binding and standard histochemical properties of all the pigments were evaluated semi-quantitatively and blind. The results indicated that mannose was in general the most consistently detected sugar residue in lipofuscin granules of humans and rats, and that this pigment may also contain acetylglucosamine, acetylgalactosamine, sialic acid, galactose and fucose. However, notable differences were found not only in the lipofuscin saccharide components of different cell types of humans and rats, but also in those in the same type of cells in both species. Although mannose was not detected in the hepatic ceroid of choline-deficient rats, this saccharide moiety was almost always present in the other ceroid pigments. Each of the ceroids also contained other types of saccharides although the frequency of the latter varied between different ceroid pigments. While lipofuscin and each of the ceroid pigments showed somewhat different lectin binding patterns, the variability in the frequency of reactivity to lectins suggests that these patterns may not be permanent but transient. In this sense, it appears that lectin histochemistry may not allow these pigments to be differentiated. Furthermore, the extractive procedures used in this study did not enable us to determine whether the saccharides detected in the pigments in situ corresponded to glycolipids or glycoproteins.

show abstract

Section: Introductioncontrasting

confidence: 85%

Lectin histochemistry of lipofuscin and certain ceroid pigments

Monserrat

Benavides

Berra

et al. 1995

Histochem Cell Biol

View full text Add to dashboard Cite

show abstract

“…Lipofuscin granules, isolated from human brains, were studied and the presence of mannose, glucose, galactose and their amine complexes was reported [55,56], Using concanavalin A, mannose and glucose residues can also be detected in human lipofuscin within lysosomes [57,58],…”

Section: Other Lipofiiscin-related Carbohydratesmentioning

confidence: 99%

Studies on Age Pigments Evolving Into a New Theory of Biological Aging

Yin¹

1995

Gerontology

View full text Add to dashboard Cite

A variety of age pigment-like fluorophores have been recognized, identified and investigated during the past three decades. They are mainly the end-products of various side-reactions of essential biological processes. Among these, the lipid peroxidation-related fluorophores formed via aldehyde-protein crosslinking are of general importance. Fluorescent advanced glycation end-products formed during glycation/Maillard reactions, are oxygen independent, carbohydrate-associated age pigment-like substances. Age pigments, particularly those identified in retinal pigment epithelium, represent another type of age pigment that originates from polyenic biomolecules, including retinoids and carotenoids. Various αβ-unsaturated aldehydes can also react with the amino groups of nucleotides to induce biological alteration. Although age pigments can be produced from different types of biological materials, the crosslinking of carbonyl and amino compounds is a common toxiferous process during biological life. In the context of various aging phenomena and degenerative diseases, this process may constitute an essential mechanism of aging --- the carbonyl toxification process of biological aging.

show abstract

“…Their structures represent a mixture of catabolic metabolites of the normal glycosylation inter mediates. However, only in the infantile form of the disease do the structures found suggest that this accumu lation might result from a specific glycosidase defect [20], Dol-PP-OS, which represent 1-7% of storage mate rial dry weight [15], probably account for some of the insoluble residue reported by Wolfe's group. How ever, Dol-PP-OS too are concentrated in lipofuscin and accumulate to some extent in the aging brain (Dol-PP-OS concentrations are 2-to 3-fold higher in oldage brain than in young brain).…”

Section: Dolicliol Compounds In Batten's Diseasementioning

confidence: 83%

“…How ever, free dolichol concentrations in the aging brain are even higher than are found in Batten's disease brain (dolichol concentrations are 10-to 20-fold higher in oldage brain than in young brain). Furthermore, dolichol is also greatly enriched in lipofuscin, representing 5% of dry weight [14,15]. It therefore became clear that doli chol accumulation was a non-specific secondary alter ation.…”

Section: Dolicliol Compounds In Batten's Diseasementioning

confidence: 99%

“…Dolichyl pyrophosphoryl oligosaccharides (Dol-PP-OS) containing 14 sugar residues are assembled in the endoplasmic reticulum and the entire oligosaccha ride chain is transferred to the asparagine residue of proteins within the lumen of the endoplasmic reticulum. Increases of 10-to 20-fold in brain Dol-PP-OS concen trations have been reported in all four forms of Batten's disease [15][16][17][18][19]. Their structures represent a mixture of catabolic metabolites of the normal glycosylation inter mediates.…”

Section: Dolicliol Compounds In Batten's Diseasementioning

confidence: 99%

See 1 more Smart Citation

Recent Biochemical and Genetic Advances in Our Understanding of Batten's Disease (Ceroid-Lipofuscinosis)

1991

View full text Add to dashboard Cite

Protein is the major component of the intra-lysosomal storage material which characteristically accumulates in Batten''s disease. In the late-infantile, juvenile and adult forms of the disease, and in a form affecting sheep, this protein is principally composed of a single polypeptide, subunit c of mitochondrial ATP synthase. Subunit c is not stored in the infantile form of Batten''s disease, supporting recent genetic data which suggest this is a distinct disease. Nor is subunit c found in storage material within other lysosomal storage diseases or in lipofuscin of old age. Subunit c storage, therefore, is specific for the later-onset forms of Batten''s disease and indeed may be central to their aetiology.

show abstract

Glycoconjugates in Storage Cytosomes from Ceroid-Lipofuscinosis (Batten’s Disease) and in Lipofuscin from Old-Age Brain

Cited by 11 publications

References 17 publications

Lectin histochemistry of lipofuscin and certain ceroid pigments

Lectin histochemistry of lipofuscin and certain ceroid pigments

Studies on Age Pigments Evolving Into a New Theory of Biological Aging

Recent Biochemical and Genetic Advances in Our Understanding of Batten's Disease (Ceroid-Lipofuscinosis)

Contact Info

Product

Resources

About