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M A Lovell et al.
Archives of neurology, 58(3), 392-396 (2001-03-20)
Markers of oxidative stress are increased in cerebrospinal fluid (CSF) of patients with Alzheimer disease (AD), although none of those reported are appropriate diagnostic markers because of the overlap between patients with AD and control subjects. To determine the ratio...
Mark D Girgis et al.
The Journal of surgical research, 185(1), 45-55 (2013-07-06)
Antibody-based therapeutics is a rapidly growing field. Small engineered antibody fragments demonstrate similar antigen affinity compared with the parental antibody but have a shorter serum half-life and possess the ability to be conjugated to nanoparticles. The goal of this study...
Barbara C Sonies et al.
Medicine, 84(3), 137-146 (2005-05-10)
Nephropathic cystinosis is a rare, autosomal recessive lysosomal storage disorder caused by mutations in the CTNS gene that codes for a cystine transporter in the lysosomal membrane. Affected patients store 50-100 times the normal amounts of cystine in their cells...
Hiroo Yoshida et al.
Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 998-999, 88-96 (2015-07-18)
The analysis of human plasma free amino acids is important for diagnosing the health of individuals, because their concentrations are known to vary with various diseases. The development of valid, reliable, and high-throughput analytical methods for amino acids analysis is...
Federico N Soria et al.
The Journal of clinical investigation, 124(8), 3645-3655 (2014-07-19)
During brain ischemia, an excessive release of glutamate triggers neuronal death through the overactivation of NMDA receptors (NMDARs); however, the underlying pathways that alter glutamate homeostasis and whether synaptic or extrasynaptic sites are responsible for excess glutamate remain controversial. Here...
E J Pappert et al.
Neurology, 47(4), 1037-1042 (1996-10-01)
To determine if ventricular cerebrospinal fluid (vCSF) alpha-tocopherol levels in Parkinson's disease (PD) patients can be increased by oral alpha-tocopherol supplementation and whether vCSF levels are linearly related to plasma alpha-tocopherol levels. In spite of its putative neuroprotective properties, alpha-tocopherol...
Valery Kh Akparov et al.
The FEBS journal, 282(7), 1214-1224 (2015-01-27)
The crystal structures of carboxypeptidase T (CpT) complexes with phenylalanine and arginine substrate analogs - benzylsuccinic acid and (2-guanidinoethylmercapto)succinic acid - were determined by the molecular replacement method at resolutions of 1.57 Å and 1.62 Å to clarify the broad...
M L Selley et al.
Neurobiology of aging, 23(3), 383-388 (2002-04-18)
There is evidence that increased blood concentrations of homocysteine may be a risk factor for Alzheimer's disease. (E)-4-hydroxy-2-nonenal (HNE) is a neurotoxic product of lipid peroxidation that is increased in the ventricular fluid and brains of patients with Alzheimer's disease....
Jian-ping Jia et al.
Chinese medical journal, 117(8), 1161-1164 (2004-09-14)
An important aspect of Alzheimer's disease (AD) is loss or impairment of cholinergic neurons. It is controversial whether there is a similar cholinergic impairment and cerebral deficit of acetylcholine (ACh) in the case of vascular dementia (VD). The purpose of...
Cyril Boulègue et al.
Antioxidants & redox signaling, 10(1), 113-125 (2007-10-27)
Native collagens are molecules that are difficult to handle because of their high tendency towards aggregation and denaturation. It was discovered early on that synthetic collagenous peptides are more amenable to conformational characterization and thus can serve as useful models...
The CARI guidelines. Kidney stones: cystine stones.
Gavin Becker
Nephrology (Carlton, Vic.), 12 Suppl 1, S4-10 (2007-02-24)
[Cystinosis: from cystine crystals to the cystinosin].
G Pintos
Nefrologia : publicacion oficial de la Sociedad Espanola Nefrologia, 23 Suppl 1, 60-70 (2003-04-24)
Martijn J Wilmer et al.
American journal of physiology. Renal physiology, 299(5), F905-F916 (2010-09-10)
Renal proximal tubules are highly sensitive to ischemic and toxic insults and are affected in diverse genetic disorders, of which nephropathic cystinosis is the most common. The disease is caused by mutations in the CTNS gene, encoding the lysosomal cystine...
Sirpa Rainesalo et al.
Neurochemical research, 29(1), 319-324 (2004-03-03)
Altered plasma and cerebrospinal fluid amino acid levels may be associated with human epilepsy. We studied three groups of patients, those with a generalized epileptic syndrome, juvenile myoclonic epilepsy, patients with refractory localization-related epilepsies, and patients with acute seizures (within...
Jie Zhang et al.
The Biochemical journal, 461(3), 497-508 (2014-05-13)
Lantibiotics are ribosomally synthesized antimicrobial peptides containing unusual amino acids. As promising alternatives to conventional antibiotics, they have a high potential for alleviating the problem of emergent antibiotic resistance, with possible applications in many industries that have antibacterial demand. Bovicin...
Wenqi Wu et al.
Urology, 83(4), 732-737 (2014-02-04)
To assess urine composition in Chinese patients with urolithiasis. Five hundred seven Chinese patients with urolithiasis from our center in southern China were included in this study. Analysis of stone composition was performed using infrared spectrometry. From all patients, 24-hour...
Je-Hung Kuo et al.
The Journal of biological chemistry, 288(19), 13522-13533 (2013-03-29)
CXCL4L1 is a highly potent anti-angiogenic and anti-tumor chemokine, and its structural information is unknown. CXCL4L1 x-ray structure is determined, and it reveals a previously unrecognized chemokine structure adopting a novel C-terminal helix conformation. The alternative helix conformation enhances the...
Holger Rumpold et al.
The Journal of clinical endocrinology and metabolism, 87(11), 5297-5303 (2002-11-05)
Age-related development of benign prostatic hyperplasia is an important health issue in developed countries. The histopathogenetic hallmark of this disease is the increase in relative and absolute numbers of smooth muscle cells (SMC). Glycoprotein hormone alpha-subunit (GPHalpha) is expressed in...
Kamran Ahmed et al.
Postgraduate medical journal, 82(974), 799-801 (2006-12-07)
Cystinuria is an autosomal recessive disorder in renal tubular and intestinal transport of dibasic amino acids, which results in increased urinary excretion of cystine, ornithine, lysine and arginine. It affects 1 in 20 000 people and is caused by a...
J Kubilus et al.
Biochimica et biophysica acta, 578(2), 484-492 (1979-06-19)
Bovine and human epidermal cells were cultured on mitomycin C treated fibroblasts. The cells were carried through four passages and found to synthesize fibrous proteins and insoluble cell envelopes. Acid buffer soluble fibrous protein, prekeratin, and urea soluble fibrous protein...
J-M Serot et al.
Journal of neurology, neurosurgery, and psychiatry, 76(11), 1585-1587 (2005-10-18)
Homocysteine may be involved in the pathogenesis of late onset Alzheimer's disease. It is implicated in the metabolism of several important pathways in the brain. Methylmalonic acid (MMA) is related to the metabolism of branched chained amino acids and fatty...
Shalini Iyer et al.
The FEBS journal, 278(22), 4304-4322 (2011-09-16)
The cystine-knot motif, made up of three intertwined disulfide bridges, is a unique feature of several toxins, cyclotides and growth factors, and occurs in a variety of species, including fungi, insects, molluscs and mammals. Growth factor molecules containing the cystine-knot...
Tadayuki Tsujita et al.
Molecular and cellular biology, 34(20), 3800-3816 (2014-08-06)
Liver-specific Nrf1 (NF-E2-p45-related factor 1) knockout mice develop nonalcoholic steatohepatitis. To identify postnatal mechanisms responsible for this phenotype, we generated an inducible liver-specific Nrf1 knockout mouse line using animals harboring an Nrf1(flox) allele and a rat CYP1A1-Cre transgene (Nrf1(flox/flox)::CYP1A1-Cre mice)....
Indrani Sinha-Hikim et al.
The journals of gerontology. Series A, Biological sciences and medical sciences, 68(7), 749-759 (2013-03-06)
Oxidative stress increases with age and is postulated to be a major causal factor for sarcopenia in aging. Here, we examined whether the administration of a cystine-based antioxidant (F1) can alleviate/delay age-specific changes in skeletal muscles. C57BL6 male mice aged...
Fredric L Coe et al.
The Journal of clinical investigation, 115(10), 2598-2608 (2005-10-04)
About 5% of American women and 12% of men will develop a kidney stone at some time in their life, and prevalence has been rising in both sexes. Approximately 80% of stones are composed of calcium oxalate (CaOx) and calcium...
Philip J Hogg
Nature reviews. Cancer, 13(6), 425-431 (2013-05-11)
Protein action in nature is generally controlled by the amount of protein produced and by chemical modification of the protein, and both are often perturbed in cancer. The amino acid side chains and the peptide and disulphide bonds that bind...
Daniel M Asplin et al.
The Journal of urology, 189(6), 2147-2151 (2012-12-25)
Pharmacological therapy for cystinuria consists of alkali salts to increase urine pH and thiol drugs to form soluble cysteine-drug complexes. The effect of alkalinizing urine on thiol drug activity has not been well studied. Urine samples were obtained from 5...
Valerio Leoni et al.
Clinical chemistry and laboratory medicine, 42(2), 186-191 (2004-04-06)
24S-Hydroxycholesterol (24OHC) and 27-hydroxycholesterol (27OHC) are two structurally similar oxysterols of different origins--the former almost exclusively formed in the brain and the latter formed to a lesser extent in the brain than in most other organs. HYPOTHESIS TO BE TESTED:...
Marrit Putker et al.
Molecular cell, 49(4), 730-742 (2013-01-22)
Forkhead box O (FOXO; DAF-16 in worms) transcription factors, which are of vital importance in cell-cycle control, stress resistance, tumor suppression, and organismal lifespan, are largely regulated through nucleo-cytoplasmic shuttling. Insulin signaling keeps FOXO/DAF-16 cytoplasmic, and hence transcriptionally inactive. Conversely...
Margaret Park et al.
Journal of the American Society of Nephrology : JASN, 13(12), 2878-2887 (2002-11-22)
Nephropathic cystinosis is a lethal disorder of lysosomal cystine storage due to defective lysosomal cystine transport. How lysosomal cystine causes this multisystemic disorder culminating in end-stage renal disease is not known, because the cystine is isolated from cellular metabolism by...
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