Showing 1-30 of 154 results for "30200"
F Rousaud et al.
Archivos espanoles de urologia, 54(9), 989-996 (2002-01-16)
Cystine renal stone is the only clinical consequence of cystinuria, an autosomal recessive hereditary disease that affects an average of 1 out of 7,000 newborns, and whose geographical distribution varies significantly. The diagnosis and treatment of this condition is reviewed...
T Peters et al.
Human molecular genetics, 12(17), 2109-2120 (2003-08-19)
Cystinuria, one of the most common inborn errors of metabolism in humans, accounts for 1-2% of all cases of renal lithiasis. It is caused by defects in the heterodimeric transporter system rBAT/b0,+AT, which lead to reduced reabsorption of cystine and...
Beatrice Bocca et al.
Annali dell'Istituto superiore di sanita, 41(2), 197-203 (2005-10-26)
The haematic concentration of 26 metals and the oxidative damage in 60 patients (20 males and 40 females) affected by Alzheimer's disease and 44 healthy individuals (33 males and 11 females) were compared. In patients, the following significant (p <...
Orna Avsian-Kretchmer et al.
Molecular endocrinology (Baltimore, Md.), 18(1), 1-12 (2003-10-04)
TGF-beta family proteins with a cystine knot motif serve as ligands for diverse families of plasma membrane receptors. Bone morphogenetic protein (BMP) antagonists represent a subgroup of these proteins, some of which bind BMPs and antagonize their actions during development...
Susan T Stephenson et al.
The Journal of allergy and clinical immunology, 136(2), 454-461 (2015-03-10)
The mechanisms underlying glucocorticoid responsiveness are largely unknown. Although redox regulation of the glucocorticoid receptor (GR) has been reported, it has not been studied in asthmatic patients. We characterized systemic cysteine oxidation and its association with inflammatory and clinical features...
Tine N Vinther et al.
Protein science : a publication of the Protein Society, 22(3), 296-305 (2013-01-03)
Insulin is a key hormone controlling glucose homeostasis. All known vertebrate insulin analogs have a classical structure with three 100% conserved disulfide bonds that are essential for structural stability and thus the function of insulin. It might be hypothesized that...
K J Bär et al.
Neurobiology of aging, 24(2), 333-338 (2002-12-25)
Increasing evidence suggests an interaction of oxidative stress and the formation of advanced glycation end products (AGE) in the onset and progression of Alzheimer's disease. We studied levels of pentosidine and N(epsilon)-(carboxymethyl)-lysine (CML) in serum and cerebrospinal fluid (CSF) of...
Julie C Lim et al.
Experimental eye research, 116, 219-226 (2013-09-24)
In this study we have sought to identify whether cystine uptake mechanisms previously identified in the rat lens are also found in the human lens. Using a combination of reverse transcriptase PCR, Western blotting and immunohistochemistry, we show that the...
Hanayo Ueoka-Nakanishi et al.
The FEBS journal, 280(14), 3220-3231 (2013-04-26)
Thioredoxin (Trx) is a key player in redox homeostasis in various cells, modulating the functions of target proteins by catalyzing a thiol-disulfide exchange reaction. Target proteins of cytosolic Trx-h of higher plants were studied, particularly in the plasma membrane, because...
Michael Thiele et al.
Journal of immunology (Baltimore, Md. : 1950), 195(5), 2343-2352 (2015-07-26)
Macrophage migration inhibitory factor (MIF), a proinflammatory cytokine and counterregulator of glucocorticoids, is a potential therapeutic target. MIF is markedly different from other cytokines because it is constitutively expressed, stored in the cytoplasm, and present in the circulation of healthy...
Arisa Higa et al.
Molecular and cellular biology, 34(10), 1839-1849 (2014-03-19)
ATF6α, a membrane-anchored transcription factor from the endoplasmic reticulum (ER) that modulates the cellular response to stress as an effector of the unfolded-protein response (UPR), is a key player in the development of tumors of different origin. ATF6α activation has...
Tian Wang et al.
Journal of pharmaceutical and biomedical analysis, 102, 519-528 (2014-12-03)
Disulfide reduction in therapeutic monoclonal antibodies can occur during cell harvest operations as a result of cell breakage. Understanding these product quality changes and manufacturers' ability to control them would likely be of concern to regulatory bodies. To study the...
Ajmila Islam et al.
Nutrition and cancer, 67(8), 1283-1292 (2015-10-17)
We previously found red wheat more effective than white wheat in reducing colon cancer risk in rats when fed during initiation and postinitiation stages. Here we examine the effect of wheat on colon cancer risk in early and late postinitiation...
Monika Stegmann et al.
European journal of immunology, 43(1), 15-21 (2012-12-13)
Selected disulfide bonds in membrane proteins are labile and are thus susceptible to changes in redox potential and/or the presence of thiol isomerase enzymes. Modification of these disulfide bonds can lead to conformational changes of the protein that in turn...
P A LeWitt et al.
Neurology, 42(11), 2111-2117 (1992-11-01)
We used two analytic methods (a multichannel coulometric electrode array with high-performance liquid chromatography, and gas chromatography-mass spectrophotometry) to measure CSF dopamine (DA) and its metabolites in mildly affected, unmedicated subjects with Parkinson's disease (PD). The mean (+/- SD) concentration...
Liyun Zhang et al.
Journal of biological inorganic chemistry : JBIC : a publication of the Society of Biological Inorganic Chemistry, 18(2), 277-286 (2013-01-24)
The purpose of this work is to characterize the interactions of two disulfide-constrained cyclic tetrapeptides [c(Ac-Cys-Pro-Phe-Cys-NH(2)), SS1; c(Ac-Cys-Pro-Gly-Cys-NH(2)), SS2] with Cu(2+) ions in order to facilitate the design of cyclic peptides as sensors for metal ions. The Cu(2+)-peptide complex cations...
Aubérie Parent et al.
Nature communications, 6, 5686-5686 (2015-01-20)
Friedreich's ataxia is a severe neurodegenerative disease caused by the decreased expression of frataxin, a mitochondrial protein that stimulates iron-sulfur (Fe-S) cluster biogenesis. In mammals, the primary steps of Fe-S cluster assembly are performed by the NFS1-ISD11-ISCU complex via the...
T Pérez-Ruiz et al.
The Analyst, 117(6), 1025-1028 (1992-06-01)
A flow injection configuration is proposed for the determination of L-cysteine and L-cystine individually and for mixtures of both analytes. The procedure is based on the rapid oxidation of L-cysteine by thallium(III) with concomitant formation of fluorescent thallium(I). The inclusion...
Young-Mi Go et al.
Critical reviews in biochemistry and molecular biology, 48(2), 173-181 (2013-01-30)
Rapid advances in redox systems biology are creating new opportunities to understand complexities of human disease and contributions of environmental exposures. New understanding of thiol-disulfide systems have occurred during the past decade as a consequence of the discoveries that thiol...
S Engelborghs et al.
Neurochemical research, 28(8), 1145-1150 (2003-07-02)
To study changes in amino acid metabolism and biogenic amines in Parkinson's disease, we set up a prospective study and measured biogenic amines, their main metabolites, and 22 different amino acids, in cerebrospinal fluid of Parkinson's disease patients (n =...
Zhimin Feng et al.
The FEBS journal, 280(14), 3365-3375 (2013-05-11)
Previously, we reported that human β-defensin (hBD)-3 can both antagonize CXCR4 function on T cells and promote receptor internalization in the absence of activation. In the present study, we explored the important structural elements of hBD-3 that are involved in...
Bulent Onal et al.
The Journal of urology, 190(1), 234-237 (2013-01-15)
We determined the effectiveness of percutaneous nephrolithotomy in children with cystine stones and present the long-term outcomes. We reviewed the data of 65 renal units in 51 children who underwent percutaneous nephrolithotomy for cystine stones between 2000 and 2012. Of...
Mehdi Irani et al.
Biochemistry, 52(7), 1280-1289 (2013-01-22)
Molecular dynamics simulations have been performed on lipase B from Candida antarctica (CalB) in its native form and with one or two oxidized residues, either methionine oxidized to methionine sulfoxide, tryptophan oxidized to 5-hydroxytryptophan, or cystine oxidized to a pair...
Pegah Saremirad et al.
Journal of chromatography. A, 1370, 147-155 (2014-12-03)
Size exclusion chromatography has been demonstrated as an effective method for refolding a variety of proteins. However, to date process development mainly relies on laboratory experimentation of individual factors. A robust model is essential for high-throughput process screening and optimization...
Hanne Sørup Tastesen et al.
PloS one, 9(11), e112859-e112859 (2014-11-13)
Low-protein and high-protein diets regulate energy metabolism in animals and humans. To evaluate whether different dietary protein sources modulate energy balance when ingested at average levels obesity-prone male C57BL/6J mice were pair-fed high-fat diets (67 energy percent fat, 18 energy...
Anne C Conibear et al.
The Journal of biological chemistry, 288(15), 10830-10840 (2013-02-23)
θ-Defensins are ribosomally synthesized cyclic peptides found in the leukocytes of some primate species and have promising applications as antimicrobial agents and scaffolds for peptide drugs. The cyclic cystine ladder motif, comprising a cyclic peptide backbone and three parallel disulfide...
G Shanker et al.
Journal of neuroscience research, 66(5), 998-1002 (2001-12-18)
Maintenance of appropriate intracellular glutathione (GSH) levels is crucial for cellular defense against oxidative damage. A suggested mechanism of methylmercury (MeHg) neurotoxicity implicates the involvement of oxygen radical formation and a decrease in cellular levels of GSH. Astrocytes play an...
M Naoi et al.
Mechanisms of ageing and development, 111(2-3), 175-188 (2000-02-03)
Dopamine neurons in the substantia nigra of human brain are selectively vulnerable and the number decline by aging at 5-10% per decade. Enzymatic and non-enzymatic oxidation of dopamine generates reactive oxygen species, which induces apoptotic cell death in dopamine neurons....
Yaoqing Diana Liu et al.
Protein science : a publication of the Protein Society, 23(12), 1753-1764 (2014-09-30)
Human IgG2 consists of disulfide-mediated structural isoforms, classified by the number of Fab arms disulfide-linked to the heavy chain hinge. In the IgG2-B isoform, both Fab arms are linked to the hinge region, and in IgG2-A, neither Fab arm are...
Y Miura
The Kurume medical journal, 47(4), 267-272 (2001-02-24)
Plasma beta-phenylethylamine (PEA) levels were determined in 27 patients with Parkinson's disease (PD) in order to evaluate its relation with the severity or clinical course of PD. The plasma PEA concentrations in PD patients were significantly lower (mean +/- SD...

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