Abstract
We concluded that Ohrid trout presented a pigment composition in liver macrophages that differed from other fish, including salmonids, where most liver phagocytes essentially display melanin. Our quantitative data support interspecies differences in the amount of liver macrophages and also that afte...
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PMID: 18767051
PDF is available here.
Abstract
The most striking morphologic change in neurons during normal aging is the accumulation of autophagic vacuoles filled with lipofuscin or neuromelanin pigments. These organelles are similar to those containing the ceroid pigments associated with neurologic disorders, particularly in diseases caused b...
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PMID: 18384642
PDF is available here.
Abstract
The canine TPP1 mutation results in progressive vision loss and retinal degeneration similar to that which occurs in human late infantile NCL. With the canine model, the natural history of disease progression in the retina provides a better understanding of the pathologic course of the disease and p...
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PMID: 18344450
PDF is available here.
Abstract
Our results demonstrated that the biomaterials from different tissues have a similar fate under accelerated oxidative/carbonyl stresses but may be differentiated by a fluorescence intensity ratio....
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PMID: 18568984
PDF is available here.
Abstract
Cerebral lipopigments (LPs)--lipofuscin and ceroid--represent a significant marker in postmitotic normal and pathologic aging, connected with causal and associated neuropathologic damage. Therefore, LP processing, lysis, and elimination may be the main targets in anti-aging and rejuvenation therapie...
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PMID: 16804015
PDF is available here.
Abstract
We will try to answer the following questions: (1) What is lipofuscin and ceroid? (2) What contributes to the accumulation of this storage material in one or the other? (3) Does this material have an effect on cellular function? Studying parallels between the accumulation of lipofuscin and ceroid ma...
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PMID: 16455164
PDF is available here.
Abstract
Fluorescent characteristics of age pigment-related materials were re-examined with improved techniques. A series of fluorescent colors, from blue to yellow-red were observed in artificial ceroid/lipofuscin. A front-surface accessory attached to a spectrofluorometer was found very useful in studying...
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PMID: 16480845
PDF is available here.
Abstract
Neuronal ceroid lipopofuscinosis (Batten disease, NCL) represents a group of common childhood neurodegenerative diseases with a shared feature of deposition of abnormal metabolic products in neurons and other tissues, including peripheral blood lymphocytes. In most forms of NCL no specific enzyme de...
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PMID: 17090516
PDF is available here.
Abstract
We have now characterized the CNS of these mice at 12 months of age. With the exception of the thalamus, Cln3Deltaex7/8 homozygotes displayed no significant regional atrophy, but a range of changes in individual laminar thickness that resulted in variable cortical thinning across subfields. Stereolo...
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PMID: 16006136
PDF is available here.
Abstract
We focus on the influence of oxidative stress on protein turnover, protein aggregate formation and the various interactions of protein aggregates with the proteasome. Furthermore, the formation and effects of protein aggregates during aging and neurodegeneration will be highlighted....
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PMID: 15325589
PDF is available here.
Abstract
We describe a new hereditary cerebellar cortical degeneration in eight adult American Staffordshire and Pit Bull Terriers. The neuronal degeneration in these animals not only affects Purkinje cells of the cerebellum but also certain thalamic nuclei. In addition, nerve cell loss appears to be associa...
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PMID: 15365721
PDF is available here.
Kumi K Takahashi,
Takashi T Ishida,
Go G Ogura,
Taeko T Ishii,
Kengo K Oshima,
Suguru S Sato,
Miho M Muroi,
Kenya K Kanazawa,
Junpei J Saito,
Yoshinori Y Otsuka,
Kazuo K Watanabe,
Makoto M Handa and
Mitsuru M Munakata
Abstract
A 65-year-old man was admitted to our hospital because of dyspnea on exertion. He had oculocutaneous albinism innately and his parents were consanguineous. His chest roentgenogram on admission showed reticulo-nodular infiltrates and cystic changes throughout both lung fields, and 7 cm mass in the le...
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PMID: 15575250
PDF is available here.
Abstract
We found the number of neurons with autofluorescent pigments had increased to 30.38% of the total in DM compared to 8.98% in the control group, and 25.36% in the aged rats. The area of autofluorescence within those neurons was 16.84% in aged rats, 13.02% in DM and 4.45% in the controls. Thus, DM cau...
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PMID: 14746930
PDF is available here.
Abstract
Focal intraplaque microhemorrhages initiate platelet and erythrocyte phagocytosis, leading to iron deposition, macrophage activation, ceroid production, and foam cell formation. Neovascularization, besides supplying plaques with leukocytes and lipoproteins, can thus promote focal plaque expansion wh...
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PMID: 12615689
PDF is available here.
Abstract
We observed urinary tract abnormality in the younger HPS sister and a porencephalic cyst in the older HPS sister; both of these developmental defects have been reported in the Cross syndrome (or oculocerebral hypopigmentation syndrome). It seems that in our patients, an overlapping of the phenotypic...
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PMID: 12827064
PDF is available here.
Abstract
A case of ceroid granuloma of the uterine cervix in a 58-year-old woman is presented, the fourth such case in the literature. It was an incidental finding during a routine pelvic examination and appeared as an exophytic brownish lesion on the anterior wall of the uterine cervix. On histological exam...
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PMID: 11917231
PDF is available here.
Abstract
Although during the normal aging process there are numerous pigmentary changes, the best recognized are those of melanin and lipofuscin. Melanin may increase (e.g., age spots, senile lentigo, or melanosis coli) or decrease (e.g., graying of hair or ocular melanin) with age, while lipofuscin (also ca...
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PMID: 11976186
PDF is available here.
Abstract
We report here that two such drugs, phosphocysteamine and N-acetylcysteine, disrupt thioester linkages in a model thioester compound, [14C]palmitoyl approximately CoA. Most importantly, in lymphoblasts derived from INCL patients, phosphocysteamine, a known lysosomotrophic drug, mediates the depletio...
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PMID: 11283676
PDF is available here.
Abstract
The term "neuronal ceroid lipofuscinosis" (NCL) describes a complex of inherited neurodegenerative conditions associated with storage of lipopigments in brain tissue. In 1989 Dyken proposed a classification of NCL based on the age, clinical symptoms, and ultrastructural aspects of the lipopigments....
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PMID: 11297320
PDF is available here.
Abstract
Demersal fish, Solea ovata, were sampled from a reference site and a site where highly contaminated sediment is dumped. Sexually immature fish from the contaminated site exhibited significantly higher EROD activity compared with counterparts sampled from the reference site. No significant difference...
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PMID: 11586770
PDF is available here.
Abstract
We report here on a retinoid model of the phenomenon discovered in 1980, in which ceroid-lipofuscin cytosomes (CLC) increased their intrinsic fluorescence when exposed to fluorescence-exciting (lambda(ex)=365nm) ultraviolet (UV) light. We modeled this effect in vitro, irradiating a methanolic soluti...
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PMID: 11113611
PDF is available here.
Abstract
We have studied the effects of hyperoxia and of cell loading with artificial lipofuscin or ceroid pigment on the postmitotic aging of human lung fibroblast cell cultures. Normobaric hyperoxia (40% oxygen) caused an irreversible senescence-like growth arrest after about 4 wk and shortened postmitotic...
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PMID: 10928983
PDF is available here.
Abstract
Hepatic lesions in 25 sika deer (Cervus nippon Temminck) aged 1-15 days, affected by selenium-deficiency cardiomyopathy, were examined histopathologically. Characteristic pathological findings, induced by stagnation of the plasma proteins of the cytoplasm, consisted of vacuolar degeneration of hepat...
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PMID: 10906259
PDF is available here.
Abstract
Vision loss was the first subjective symptom of the disease in all 12 cases. Among these cases, nine of 12 patients (75%) developed neurologic deficits an average of 3 years after the onset of visual deterioration. CONCLUSION: Because visual symptoms usually precede neurologic dysfunction, JNCL shou...
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PMID: 10870925
PDF is available here.
Abstract
We hypothesize that large amounts of undegradable ceroid/lipofuscin within the acidic vacuolar compartment may interfere with lysosomal function, resulting in poor renewal of long-lived proteins and worn-out/damaged organelles, decreased adaptability, and cell death....
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PMID: 10673148
PDF is available here.
Abstract
The clinicopathologic features of 31 surgical patients with xanthogranulomatous cholecystitis were analysed in relation to the immunohistochemical demonstration of bacterial antigens using a polyclonal anti-E. coli antibody. The time period after the initial clinical manifestation was critical for i...
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PMID: 10571816
PDF is available here.
Abstract
These results suggest that macrophages might phagocytize Leydig cells, and store their digested materials as ceroid-like pigment....
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PMID: 10563302
PDF is available here.
Abstract
We hypothesize that the enhanced sensitivity of ceroid/lipofuscin-loaded cells to oxidative stress may be caused by the increased amounts of lysosomal enzymes, known as mediators of oxidative damage, and/or by catalysis of intralysosomal oxidative reactions by lipofuscin-associated iron....
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PMID: 10579636
PDF is available here.
Abstract
Different stages of atherosclerosis were studied by immunohistochemistry on whole-mount longitudinal sections of carotid endarterectomy specimens. In the adaptive intimal thickening the predominant cell type were smooth muscle cells. The fatty streaks contained both smooth muscle cells and macrophag...
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PMID: 10690346
PDF is available here.
Abstract
The mechanisms involved in the accumulation of ceroid/lipofuscin within non-dividing cells are not totally understood. Oxidative stress, as well as diminished activity of lysosomal proteolytic enzymes, are known to induce ceroid/lipofuscin accumulation in a variety of cell types. In order to clarify...
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PMID: 9818731
PDF is available here.
Abstract
The presence of ceroid, a complex of protein associated with oxidized lipids, is commonly observed in human atherosclerotic lesions. When the human aortic walls were examined by Perls' staining, it was found that the iron deposits were evident in aortas with atherosclerosis. The extent of iron depos...
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PMID: 9690911
PDF is available here.
Abstract
We report here that a short-term exposure of cultured neonatal rat cardiac myocytes to the thiol protease-inhibitor leupeptin, causes an accumulation of numerous electron-dense autophagic lysosomes within the cells. Although very similar to LF by ultrastructure, these inclusions do not display LF-sp...
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PMID: 9541135
PDF is available here.
Abstract
Hermansky-Pudlak Syndrome (HPS) is a rare, inheritable disorder characterized by the classic triad of oculo-cutaneous albinism, platelet dysfunction, and ceroid deposition. An associated complication is pulmonary fibrosis with progressive restrictive lung disease. This report discusses the lung invo...
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PMID: 9869831
PDF is available here.
Abstract
Recent studies have consistently shown that, during oxidative damage, glycation, and other oxygen stress-related reactions, various biomolecules are converted into ceroid- and lipofuscin-like fluorescent pigments. In this study, artificial ceroid/lipofuscin was produced by exposing rat liver fractio...
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PMID: 9430105
PDF is available here.
Abstract
The significant increase of apoptotic bodies in the lining colonic epithelium indicated that this type of cell death is not due to the natural programmed cell renewal, but to the action of laxatives. Because the autofluorescent pigment of melanosis coli contains melanin (as well as glycoconjugates)...
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PMID: 9283862
PDF is available here.
Abstract
These results suggest that fibrosis and fatty droplet deposition lead to microvascular heterogeneity. Therefore, the degree and distribution of fatty droplets, ceroid-lipofuscinosis, and intrasinusoidal neutrophil infiltration differ, depending on the etiology of fatty liver, and are an important hi...
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PMID: 8986240
PDF is available here.
Abstract
Two biopsy specimens showed unusual histology characterized by features consistent with resolving oral lichen planus and voluminous, finely granular cells in the lamina propria, resembling the cells of granular cell tumour. Immunocytochemistry and lipid-pigment histochemistry indicated that the gran...
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PMID: 8887082
PDF is available here.
Abstract
Serious studies of the formation mechanisms of age-related pigments and their possible cellular influence have been hampered for a long time by discrepancies and controversies over the definition, fluorescence emission, origin, and composition of these pigments. This review discusses several critica...
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PMID: 8902532
PDF is available here.
Abstract
The Hermansky-Pudlak Syndrome (HPS) is a rare autosomal recessively inherited triad of oculocutaneous albinism hemorrhagic diathesis and accumulation of ceroid in tissues. This article comprising the published reports of 232 patients is extended by the personal observation of a 7-year-old boy with H...
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PMID: 8901188
PDF is available here.
Abstract
An electron microscopic study of peripheral leukocytes obtained from a 39 year old woman with Hermansky-Pudlak syndrome was performed. Ceroid pigment granules were found within the lysosomes in 3.5% of monocytes and 5.4% of lymphocytes. Infrequently, pigment granules were also found in the parallel...
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PMID: 8581150
PDF is available here.
Abstract
Three cases of ceroid granulomas of the female genital system are presented, involving the cervix in two and lesions in the ovaries and bowel serosa in the other. Ceroid granulomas are unusual and interesting lesions formed when suitable substrates accumulate within macrophages to such an extent tha...
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PMID: 8543632
PDF is available here.
Abstract
Structural and chemical modifications of plasma lipoproteins retained in atherosclerotic lesions, especially LDL, are a characteristic of atherogenesis. The major cholesterol-containing structures believed to be derived primarily from LDL are monomeric or aggregated native or modified LDL particles,...
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PMID: 8520855
PDF is available here.
Abstract
A prominent feature of human atherosclerosis is the lipid-laden foamy macrophage, which often also contains the insoluble pigment, ceroid. The culture of macrophage-like cells, P388D1s, with artificial lipoproteins composed of cholesteryl linoleate (CL) and bovine serum albumin (BSA) results in foam...
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PMID: 7581821
PDF is available here.
Abstract
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...
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PMID: 7584550
PDF is available here.
Abstract
We report an autopsy case (39y, male), who died of pulmonary fibrosis and hemorrhage, with analysis of auto-fluorescent ceroid-like pigments (CLP). Pigmented macrophages were seen in almost all organs, especially marked in bone marrow, spleen, liver, colon, lymph nodes and kidneys. Ultrastructurally...
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PMID: 8821340
PDF is available here.
Abstract
Lipofuscin pigment (LP) accumulations and ceroid pigment (CP) storages were demonstrated by multiple consensus studies. On the contrary, fewer researches, sometimes with opposite conclusions were made on brain LP and CP decrease, dissolution and elimination. Neuroactive agents (such as Meclofenoxate...
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PMID: 8821338
PDF is available here.
Abstract
Six piglets, aged 4 weeks (weaning) at the beginning of the experiment, were fed a diet lacking antioxidants (vitamin E and selenium) and enriched with oxidated cod liver oil (the peroxide value of which was 112.54 meqv/kg). Four of the experimental pigs were killed for necropsy at the age of 2 mont...
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PMID: 8821341
PDF is available here.
Abstract
We propose the hypothesis that the production of superoxide by neutrophils causes further lipid peroxidation of native LDL and then produces large amounts of oxidatively modified LDL which is the souse of ceroid pigment accumulated within the foam cells in human atherosclerotic lesions....
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PMID: 8821320
PDF is available here.
Abstract
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-prote...
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PMID: 8821329
PDF is available here.
Abstract
Necropsy samples of atherosclerotic lesions of different histological stages have been analysed. Ceroid was present in all the lesions, within lipid-laden macrophage foam cells and extracellularly in the atheromatous core of advanced lesions. Mean levels of 7 beta-hydroxycholesterol, 26-hydroxychole...
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PMID: 8821321
PDF is available here.
Abstract
Visual acuities ranged from 20/50 to 5/200. All patients had nystagmus. Forty-three patients had strabismus; esotropia was found in 24 patients; exotropia in 18 patients; and one patient had hypertropia. Posterior embryotoxon occurred in 15 patients and Axenfeld anomaly in 4 patients. Iris pigmentat...
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PMID: 8719678
PDF is available here.
Abstract
Flow cytometry can be used to quantify the accumulation of ceroid in macrophages, the result of cellular handling of certain lipoproteins. Using P388D1 cells, a murine-derived macrophage-like cell line, the effect of the lipophilic antioxidant, DL-alpha-tocopherol, upon the uptake and accumulation o...
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PMID: 7811710
PDF is available here.
Abstract
Routine histology of hares caught for ecological monitoring showed extracellular curvilamellar ceroid in a focal degeneration process of reserve adipose tissue in 19 animals. The defect was localized most frequently in parametrial adipose tissue, in mesovary, close to suprarenals and in spleen hilus...
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PMID: 7923424
PDF is available here.
Abstract
Our observations are discussed in the context of the observed increase in the severity of atherosclerosis in diabetes....
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PMID: 8198540
PDF is available here.
Abstract
The accumulation in macrophages of ceroid, an autofluorescent polymer composed of oxidised protein and lipid, can be monitored semiquantitatively by staining techniques. However, such methods are crude and give little information about the amount of ceroid within cells. Flow cytometry, however, can...
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PMID: 8026227
PDF is available here.
Abstract
Studies using 125I-low density lipoprotein (125I-LDL) show that probucol (10 microM) and alpha-tocopherol (100 microM) inhibit protein degradation in LDL exposed to Cu (II) in vitro. The inhibitory effect of alpha-tocopherol on protein fragmentation exceeded that of probucol. On the other hand, prob...
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PMID: 8019642
PDF is available here.