نتایج جستجو برای: lipidosis

تعداد نتایج: 260  

Journal: :The Journal of biological chemistry 2006
Angel Baldán, Paul Tarr, Charisse S Vales, Joy Frank, Thomas K Shimotake, Sam Hawgood, Peter A Edwards,

We show that mice lacking the ATP-binding cassette transmembrane transporter ABCG1 show progressive and age-dependent severe pulmonary lipidosis that recapitulates the phenotypes of different respiratory syndromes in both humans and mice. The lungs of chow-fed Abcg1(-/-) mice, >6-months old, exhibit extensive subpleural cellular accumulation, macrophage, and pneumocyte type 2 hypertrophy, massi...

Journal: :The Journal of biological chemistry 1983
M W Spence, J T Clarke, H W Cook,

The metabolism of endogenous sphingomyelin labeled with 32P or [methyl-3H]choline and of exogenous [choline-methyl-3H], [32P]-, or [N-acyl-1-14C]sphingomyelin was studied in normal and Niemann-Pick Type A (NP-A) cultured fibroblasts. Despite a greater than 96% decrease in lysosomal sphingomyelinase activity in the NP-A cells, they were able to degrade endogenously produced [32P]- or [methyl-3H]...

Journal: :Journal of lipid research 1969
D E Vance, W Krivit, C C Sweeley,

Concentrations of four neutral glycosyl ceramides were determined in plasma and erythrocytes from nine hemizygous patients with Fabry's disease (a hereditary glycolipid lipidosis), from the sister of one of the patients, and from the heterozygous mother of another one. The concentration of a trihexosyl ceramide, galactosylgalactosylglucosyl ceramide, was elevated in plasma from the patients abo...

Journal: :Journal of feline medicine and surgery 2010
Alexandre G T Daniel, Sílvia R R Lucas, Archivaldo R Júnior, Paula R G Monteiro, Daniela Ramos, Carolina G Pires, Idércio L Sinhorini,

A case of acquired skin fragility syndrome associated with hepatic disease in a 9-year-old, spayed female, domestic shorthair cat is described. The cat was admitted to the veterinary hospital of the University of São Paulo (Brazil) with a 6-week history of vomiting, inappetence and weight loss. Remarkable signs were weakness, lethargy and profound jaundice that had been present for 10 days acco...

Journal: :The Journal of Nutrition 2002
GéraldineBlanchard, Bernard MParagon, FabienMilliat, ClaudeLutton,

Journal: :Analytical and Bioanalytical Chemistry 2019
AlexDexter, Rory T.Steven, AatekaPatel, Lea AnnDailey, Adam J.Taylor, DougBall, JanKlapwijk, BenForbes, Clive P.Page, JosephineBunch,

Journal: :Journal of neurology, neurosurgery, and psychiatry 1973
A Harden, G Pampiglione, N Picton-Robinson,

Combined recordings of the electroretinogram (ERG) and the cortical visual evoked response (VER) have been made together with the electroencephalogram (EEG) in 16 children suffering from a `late infantile' form of `neuronal lipidosis'. The ERG was not usually recordable, in keeping with a gross loss of function of the retinal receptor elements. However, in all the 16 children, at whatever stage...

Journal: :Archives of disease in childhood 1969
G J Hooghwinkel, H H van Gelderen, A Staal,

Histological and chemical examinations of biopsy specimens from cerebral tissue of children suffering from undiagnosed progressive brain disease are performed increasingly. Important information is provided, which, though rarely of therapeutic value, does increase precision of diagnosis, prognosis, and genetic advice (Cumings, 1965a, b, c; Poser, 1962; Adams, 1965). This is especially true of t...

2012
Luis M. B. B. Estronca, Joao C. P. Silva, Julio L. Sampaio, Andrej Shevchenko, Paul Verkade, Alfin D. N. Vaz, Winchil L. C. Vaz, Otilia V. Vieira,

BACKGROUND Atherosclerosis starts by lipid accumulation in the arterial intima and progresses into a chronic vascular inflammatory disease. A major atherogenic process is the formation of lipid-loaded macrophages in which a breakdown of the endolysomal pathway results in irreversible accumulation of cargo in the late endocytic compartments with a phenotype similar to several forms of lipidosis....

Journal: :Journal of Human Genetics 2002
J.Ishii, M.Nagano, T.Kujiraoka, M.Ishihara, T.Egashira, D.Takada, M.Tsuji, H.Hattori, M.Emi,

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