Cysteamine inhibits lysosomal oxidation of low density lipoprotein in human macrophages and reduces atherosclerosis in mice [PDF]
, 2019 Background and aims: We have shown previously that low density lipoprotein (LDL) aggregated by vortexing is internalised by macrophages and oxidised by iron in lysosomes to form the advanced lipid/protein oxidation product ceroid.
Ahmad +73 more
core +1 more source
A new proof of evidence of cysteamine quantification for therapeutic drug monitoring in patients with cystinosis [PDF]
, 2022 Background To date, measurement of intracellular cystine is used for the therapeutic monitoring of patients affected by cystinosis in treatment with cysteamine.
Addobbati, Riccardo +6 more
core +2 more sources
Chiari I Malformation in Nephropathic Cystinosis [PDF]
The Journal of Pediatrics, 2015 To determine the relative incidence of Chiari I malformations in children with cystinosis compared with those in the general population.Magnetic resonance imaging scans were performed on 53 patients with nephropathic cystinosis and 120 controls, age range 3-18 years.Ten of 53 (18.9%) patients with cystinosis had Chiari I or tonsillar ectopia, and only ...
Kavya I. Rao +2 more
openaire +2 more sources
Altered gene expression in asymptomatic SHIV-infected rhesus macaques () [PDF]
, 2006 Simian-Human immunodeficiency virus is a chimeric virus which, in rhesus macaques (Macacca mulatta) closely imitates immunodeficiency virus infection in human (HIV). A relatively new way to study pathogenesis of viral infection is to study alterations in
Phillips Aaron T +5 more
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Ophthalmology and Therapy, 2018 Cystinosis, a rare autosomal recessive disease caused by intracellular cystine accumulation, occurs in an estimated 1/100,000–200,000 live births. Ocular non-nephropathic cystinosis is typically diagnosed during adulthood, when patients present with ...
Susmito Biswas +5 more
doaj +1 more source
Low density lipoprotein oxidation by ferritin at lysosomal pH [PDF]
, 2018 Oxidation of low density lipoprotein (LDL) has been proposed to be involved in the pathogenesis of atherosclerosis. We have previously shown that LDL can be oxidised by iron in lysosomes.
Leake, David S., Ojo, Oluwatosin O.
core +1 more source
Infantile Nephropathic Cystinosis: A Novel CTNS Mutation
Eurasian Journal of Medicine, 2019 Cystinosis is a rare autosomal recessive metabolic disorder characterized by the accumulation of cystine in lysosomes, which results from defects in the carrier-mediated transport protein encoded by the CTNS gene.
Hakan Doneray +4 more
doaj +1 more source
Differential diagnosis of (inherited) amino acid metabolism or transport disorders [PDF]
, 1992 __Abstract__ Disorders of amino acid metabolism or transport are most clearly expressed in urine. Nevertheless the interpretation of abnormalities in urinary amino acid excretion remains difficult. An increase or decrease of almost every amino acid in
Blom, W.A.M. (Wim) +1 more
core +2 more sources
Macrophage polarization impacts tunneling nanotube formation and intercellular organelle trafficking. [PDF]
, 2019 Tunneling nanotubes (TNTs) are cellular extensions enabling cytosol-to-cytosol intercellular interaction between numerous cell types including macrophages.
Cherqui, Stephanie +4 more
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Outer retinal circular structures in patients with Bietti crystalline retinopathy. [PDF]
, 2011 [Background] : Bietti crystalline retinopathy (BCR) is a distinct retinal degenerative disease characterised by retinal degeneration with many yellow–white crystals located mainly at the posterior pole area.
Guo, Congrong +7 more
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