cysteamine

Summary

Summary: A radiation-protective agent that oxidizes in air to form CYSTAMINE. It can be given intravenously or orally to treat radiation sickness. The bitartrate has been used for the oral treatment of nephropathic cystinosis.

Top Publications

  1. ncbi Cysteamine prevents inhibition of thiol-containing enzymes caused by cystine or cystine dimethylester loading in rat brain cortex
    Virginia Cielo Rech
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, UFRGS, Rua Ramiro Barcelos 2600, CEP 90 035 003, Porto Alegre, Rio Grande do Sul, Brazil
    Metab Brain Dis 23:133-45. 2008
  2. ncbi Transduced PEP-1-FK506BP ameliorates atopic dermatitis in NC/Nga mice
    So Young Kim
    Department of Biomedical Science and Research Institute of Bioscience and Biotechnology, Hallym University, Chunchon, Korea
    J Invest Dermatol 131:1477-85. 2011
  3. ncbi Target-specific gene silencing of layer-by-layer assembled gold-cysteamine/siRNA/PEI/HA nanocomplex
    Min Young Lee
    Department of Materials Science and Engineering, Pohang University of Science and Technology POSTECH, San 31, Hyoja Dong, Nam Gu, Pohang, Kyungbuk 790 784, Korea
    ACS Nano 5:6138-47. 2011
  4. pmc Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase
    Maria Borrell-Pages
    Institut Curie, CNRS UMR 146, Orsay, France
    J Clin Invest 116:1410-24. 2006
  5. pmc Cysteamine suppresses invasion, metastasis and prolongs survival by inhibiting matrix metalloproteinases in a mouse model of human pancreatic cancer
    Toshio Fujisawa
    Tumor Vaccines and Biotechnology Branch, Division of Cellular and Gene Therapies, Center for Biologics Evaluation and Research, Food and Drug Administration, Bethesda, Maryland, United States of America
    PLoS ONE 7:e34437. 2012
  6. pmc Cysteamine, the molecule used to treat cystinosis, potentiates the antimalarial efficacy of artemisinin
    Gundula Min-Oo
    Department of Biochemistry, Complex Traits Program, McGill University, 3649 Sir William Osler, Room 366, Montreal, QC H3G 0B1, Canada
    Antimicrob Agents Chemother 54:3262-70. 2010
  7. ncbi Design of a Pep-1 peptide-modified liposomal nanocarrier system for intracellular drug delivery: Conformational characterization and cellular uptake evaluation
    Myung J Kang
    Division of Pharmaceutical Sciences, College of Pharmacy, Chung Ang University, Seoul, Republic of Korea
    J Drug Target 19:497-505. 2011
  8. ncbi Potential of cystamine and cysteamine in the treatment of neurodegenerative diseases
    C Gibrat
    Centre de Recherche du CHUL CHUQ, Axe Neurosciences, 2705 Boulevard Laurier, Quebec, QC, Canada, G1V 4G2
    Prog Neuropsychopharmacol Biol Psychiatry 35:380-9. 2011
  9. ncbi Modulation of Th1/Th2 immune responses to HIV-1 Tat by new pro-GSH molecules
    Alessandra Fraternale
    Department of Biomolecular Sciences, University of Urbino Carlo Bo, Via Saffi, 2, 61029 Urbino PU, Italy
    Vaccine 29:6823-9. 2011
  10. ncbi Cysteamine therapy delays the progression of nephropathic cystinosis in late adolescents and adults
    Albane Brodin-Sartorius
    Department of Nephrology, Necker Enfants Malades Hospital, Paris, France
    Kidney Int 81:179-89. 2012

Detail Information

Publications210 found, 100 shown here

  1. ncbi Cysteamine prevents inhibition of thiol-containing enzymes caused by cystine or cystine dimethylester loading in rat brain cortex
    Virginia Cielo Rech
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, UFRGS, Rua Ramiro Barcelos 2600, CEP 90 035 003, Porto Alegre, Rio Grande do Sul, Brazil
    Metab Brain Dis 23:133-45. 2008
    ..46 micromol/g body weight of cysteamine from the 16th to the 20th postpartum day and sacrificed after 12 h...
  2. ncbi Transduced PEP-1-FK506BP ameliorates atopic dermatitis in NC/Nga mice
    So Young Kim
    Department of Biomedical Science and Research Institute of Bioscience and Biotechnology, Hallym University, Chunchon, Korea
    J Invest Dermatol 131:1477-85. 2011
    ....
  3. ncbi Target-specific gene silencing of layer-by-layer assembled gold-cysteamine/siRNA/PEI/HA nanocomplex
    Min Young Lee
    Department of Materials Science and Engineering, Pohang University of Science and Technology POSTECH, San 31, Hyoja Dong, Nam Gu, Pohang, Kyungbuk 790 784, Korea
    ACS Nano 5:6138-47. 2011
    ..In this work, a cysteamine modified gold nanoparticles (AuCM)/siRNA/polyethyleneimine (PEI)/hyaluronic acid (HA) complex was successfully ..
  4. pmc Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase
    Maria Borrell-Pages
    Institut Curie, CNRS UMR 146, Orsay, France
    J Clin Invest 116:1410-24. 2006
    ..We demonstrate that cysteamine, the FDA-approved reduced form of cystamine, is neuroprotective in HD mice by increasing BDNF levels in brain...
  5. pmc Cysteamine suppresses invasion, metastasis and prolongs survival by inhibiting matrix metalloproteinases in a mouse model of human pancreatic cancer
    Toshio Fujisawa
    Tumor Vaccines and Biotechnology Branch, Division of Cellular and Gene Therapies, Center for Biologics Evaluation and Research, Food and Drug Administration, Bethesda, Maryland, United States of America
    PLoS ONE 7:e34437. 2012
    b>Cysteamine, an anti-oxidant aminothiol, is the treatment of choice for nephropathic cystinosis, a rare lysosomal storage disease...
  6. pmc Cysteamine, the molecule used to treat cystinosis, potentiates the antimalarial efficacy of artemisinin
    Gundula Min-Oo
    Department of Biochemistry, Complex Traits Program, McGill University, 3649 Sir William Osler, Room 366, Montreal, QC H3G 0B1, Canada
    Antimicrob Agents Chemother 54:3262-70. 2010
    ..We report that cysteamine (Cys), the aminothiol used to treat nephropathic cystinosis in humans, strongly potentiates the efficacy of ..
  7. ncbi Design of a Pep-1 peptide-modified liposomal nanocarrier system for intracellular drug delivery: Conformational characterization and cellular uptake evaluation
    Myung J Kang
    Division of Pharmaceutical Sciences, College of Pharmacy, Chung Ang University, Seoul, Republic of Korea
    J Drug Target 19:497-505. 2011
    ..In conclusion, we found that the Pep1-Lipo formulation was a promising nanocarrier system for intracellular delivery of macromolecules...
  8. ncbi Potential of cystamine and cysteamine in the treatment of neurodegenerative diseases
    C Gibrat
    Centre de Recherche du CHUL CHUQ, Axe Neurosciences, 2705 Boulevard Laurier, Quebec, QC, Canada, G1V 4G2
    Prog Neuropsychopharmacol Biol Psychiatry 35:380-9. 2011
    ..several neuroprotective agents emerging from experimental animal work, cystamine, as well as its reduced form cysteamine, have been identified as potential candidate drugs...
  9. ncbi Modulation of Th1/Th2 immune responses to HIV-1 Tat by new pro-GSH molecules
    Alessandra Fraternale
    Department of Biomolecular Sciences, University of Urbino Carlo Bo, Via Saffi, 2, 61029 Urbino PU, Italy
    Vaccine 29:6823-9. 2011
    ....
  10. ncbi Cysteamine therapy delays the progression of nephropathic cystinosis in late adolescents and adults
    Albane Brodin-Sartorius
    Department of Nephrology, Necker Enfants Malades Hospital, Paris, France
    Kidney Int 81:179-89. 2012
    ..is a multisystem autosomal recessive disease caused by cystine accumulation, which is usually treated by oral cysteamine. In order to determine long-term effects of this therapy, we enrolled 86 adult patients (mean age 26...
  11. pmc Heptahelical protein PQLC2 is a lysosomal cationic amino acid exporter underlying the action of cysteamine in cystinosis therapy
    Adrien Jézégou
    Universite Paris Descartes, Sorbonne Paris Cité, Centre National de Recherche Scientifique, Unité Mixte de Recherche 8192, Centre Universitaire des Saints Peres, F 75006 Paris, France
    Proc Natl Acad Sci U S A 109:E3434-43. 2012
    ..Finally, PQLC2 transports a lysine-like mixed disulfide that serves as a chemical intermediate in cysteamine therapy of cystinosis, and PQLC2 gene silencing trapped this intermediate in cystinotic cells...
  12. ncbi Cysteamine prevents the development of lens opacity in a rat model of selenite-induced cataract
    Sang Mok Lee
    Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea
    Invest Ophthalmol Vis Sci 53:1452-9. 2012
    ..The authors investigated whether TG2 is involved in selenite-induced cataract formation in rats and whether cysteamine, a chemical inhibitor of TG2, can prevent cataract formation in this model.
  13. pmc Cysteamine attenuates the decreases in TrkB protein levels and the anxiety/depression-like behaviors in mice induced by corticosterone treatment
    Ammar Kutiyanawalla
    Department of Psychiatry and Health Behavior, Georgia Health Sciences University, Augusta, Georgia, United States of America
    PLoS ONE 6:e26153. 2011
    ..Studies designed to elucidate the mechanisms whereby TrkB signaling is regulated in chronic stress might provide valuable information for the development of new therapeutic strategies for several stress-related psychiatric disorders...
  14. ncbi Glutathione synthesis during in vitro maturation of bovine oocytes: role of cumulus cells
    D G De Matos
    Centro de Investigaciones Reproductivas Perez Companc, Buenos Aires, Argentina
    Biol Reprod 57:1420-5. 1997
    ..during IVM of bovine oocytes in the presence of cystine, cysteine, the cysteine analogue N-acetylcysteine, and cysteamine. For this purpose, cumulus-oocyte complexes (COCs), denuded oocytes (DOs), and DOs in coculture with a cumulus ..
  15. ncbi Formulation and in vitro evaluation of cysteamine hydrochloride viscous solutions for the treatment of corneal cystinosis
    Sibel Bozdag
    Department of Pharmaceutical Technology, Hacettepe University, Sihhiye, Ankara, Turkey
    Eur J Pharm Biopharm 70:260-9. 2008
    In the present study, viscous solutions of cysteamine hydrochloride (CH) were prepared by using 0.5%, 1.0%, 1.5% or 3.0% of hydroxypropylmethylcellulose (HPMC) and were evaluated for their in-vitro characteristics and stability...
  16. ncbi Pharmacological treatment of nephropathic cystinosis with cysteamine
    Robert Kleta
    NHGRI, Building 10, Room 10C 107, MSC 1851, 10 Center Drive, Bethesda, MD 20892, USA
    Expert Opin Pharmacother 5:2255-62. 2004
    ..g., swallowing disorders). Since the introduction of cysteamine into the pharmacological management of cystinosis, well-treated adolescent and young adult patients have ..
  17. ncbi Long-term follow-up of well-treated nephropathic cystinosis patients
    Robert Kleta
    Section on Human Biochemical Genetics, Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland 20892 1851, USA
    J Pediatr 145:555-60. 2004
    We report the excellent clinical outcomes of siblings with nephropathic cystinosis treated diligently with cysteamine starting at 20 months and 2 months of age...
  18. ncbi Inhibition of murine AIDS by pro-glutathione (GSH) molecules
    A Fraternale
    Institute of Biological Chemistry Giorgio Fornaini, Via Saffi, 2, University of Urbino Carlo Bo, 61029 Urbino PU, Italy
    Antiviral Res 77:120-7. 2008
    ..These results show that the administration of pro-GSH molecules may favorably substitute for the use of GSH as such...
  19. ncbi Transduced PEP-1-AMPK inhibits the LPS-induced expression of COX-2 and iNOS in Raw264.7 cells
    Min Jea Shin
    Department of Biomedical Science and Research Institute for Bioscience and Biotechnology, Hallym University Medical Center, Chunchon 200 704, Korea
    BMB Rep 43:40-5. 2010
    ..These results suggest that the PEP-1-AMPK fusion protein can be used for the protein therapy of COX-2 and NO-related disorders such as inflammation and neuronal diseases. [BMB reports 2010; 43(1): 40-45]...
  20. ncbi Cysteamine, the natural metabolite of pantetheinase, shows specific activity against Plasmodium
    Gundula Min-Oo
    Department of Biochemistry, McGill University, 3655 Promenade Sir William Osler, Montreal, QC, Canada
    Exp Parasitol 125:315-24. 2010
    ..Treatment of mice with the pantetheinase metabolite cysteamine reduces blood-stage replication of P. chabaudi and significantly increases survival...
  21. ncbi Capacity of adult and prepubertal mouse oocytes to undergo embryo development in the presence of cysteamine
    D G De Matos
    Dutch Speaking Brussels Free University, Laarbeeklaan 101, 1090 Brussels, Belgium
    Mol Reprod Dev 64:214-8. 2003
    ..The IVM medium was supplemented with: 0, 25, 50, 100, or 200 microM of cysteamine. To study the novo GSH synthesis, 5 mM BSO was added during IVM of adult or prepubertal oocyte...
  22. ncbi Effects of cysteamine on MPTP-induced dopaminergic neurodegeneration in mice
    Linjuan Sun
    Department of Neurobiology and Neurology, Key Laboratory for Neurodegenerative Diseases of Ministry of Education, Xuanwu Hospital of Capital Medical University, 45 Changchun Street, Beijing 100053, China
    Brain Res 1335:74-82. 2010
    b>Cysteamine is a degradation product of the amino acid cysteine and a reduced form of cystamine...
  23. ncbi Translocation or membrane disintegration? Implication of peptide-membrane interactions in pep-1 activity
    Sónia Troeira Henriques
    Centro de Quimica e Bioquimica, Faculdade de Ciencias da Universidade de Lisboa, Ed C8, Campo Grande, 1749 016 Lisboa, Portugal
    J Pept Sci 14:482-7. 2008
    ..In this review, the ability of pep-1 to transverse the membrane by an endocytosis-independent mechanism, not mediated by pores as well as an ability to induce membrane disintegration at high peptide concentration, is demonstrated...
  24. ncbi Attenuation of cysteamine-induced duodenal ulcer with Cochinchina momordica seed extract through inhibiting cytoplasmic phospholipase A2/5-lipoxygenase and activating γ-glutamylcysteine synthetase
    Ki Seok Choi
    Laboratory of Translational Medicine, Gachon University Lee Gil Ya Cancer and Diabetes Institute, Incheon, Korea
    J Gastroenterol Hepatol 27:13-22. 2012
    b>Cysteamine is a reducing aminothiol used for inducing duodenal ulcer through mechanisms of oxidative stress related to thiol-derived H(2)O(2) reaction...
  25. ncbi Cysteamine restores glutathione redox status in cultured cystinotic proximal tubular epithelial cells
    Martijn J Wilmer
    Laboratory of Genetic Endocrine and Metabolic Diseases, Department of Laboratory Medicine, Radboud University Nijmegen Medical Centre, The Netherlands
    Biochim Biophys Acta 1812:643-51. 2011
    ..The most common treatment involves intracellular cystine depletion by cysteamine, delaying the development of end stage renal disease by a yet elusive mechanism...
  26. ncbi Cysteamine increases expression and activity of H+-K+-ATPase of gastric mucosal cells in weaning piglets
    Zhi Min Shi
    Key Laboratory of Animal Physiology and Biochemistry, Nanjing Agricultural University, Nanjing 210095, Jiangsu Province, China
    World J Gastroenterol 11:6707-12. 2005
    To determine the in vivo and in vivo effects of cysteamine (CS) on expression and activity of H(+)-K(+)-ATPase of gastric mucosal cells in weaning piglets.
  27. ncbi Leptin is involved in the effects of cysteamine on egg laying of hens, characteristics of eggs, and posthatch growth of broiler offspring
    Y Hu
    Key Laboratory of Animal Physiology and Biochemistry, Nanjing Agricultural University, Nanjing, P R China
    Poult Sci 87:1810-7. 2008
    b>Cysteamine has been reported to modulate energy homeostasis and exert significant growth-promoting effects in broiler chickens...
  28. ncbi Radioprotection of DNA by thiols: relationship between the net charge on a thiol and its ability to protect DNA
    S Zheng
    Department of Chemistry, University of California, San Diego, La Jolla 92093
    Radiat Res 114:11-27. 1988
    ..to protect DNA was shown to depend upon the net charge (Z) at pH 7 in the order WR 1065 (Z = +2) greater than cysteamine (Z = +1) greater than 2-mercaptoethanol (Z = 0) approximately equal to dithiothreitol (Z = 0) greater than GSH (..
  29. ncbi Long-term treatment of cystinosis in children with twice-daily cysteamine
    Ranjan Dohil
    Department of Pediatrics, University of California, San Diego, La Jolla, CA, USA
    J Pediatr 156:823-7. 2010
    Cystinosis causes renal and other organ failure. Treatment with 6-hourly cysteamine bitartrate (Cystagon, Mylan, Morgantown, West Virginia) reduces intracellular cystine and the rate of organ deterioration...
  30. ncbi NAC/MEA conjugate: a new potent antioxidant which increases the GSH level in various cell lines
    J Oiry
    Laboratoire de Chimie Organique Biomoléculaire de Synthèse, UMR 5625 CNRS UM II, Universite Montpellier II, Sciences et Techniques du Languedoc, France
    Bioorg Med Chem Lett 11:1189-91. 2001
    ....
  31. ncbi Cysteamine dioxygenase: evidence for the physiological conversion of cysteamine to hypotaurine in rat and mouse tissues
    Relicardo M Coloso
    Cornell University, Division of Nutritional Sciences, Ithaca, New York 148453, USA
    Adv Exp Med Biol 583:25-36. 2006
  32. ncbi Supplementation with cysteamine during maturation and embryo culture on embryo development of prepubertal goat oocytes selected by the brilliant cresyl blue test
    Aixa Urdaneta
    Universitat Autonoma de Barcelona, 08193 Bellaterra, Barcelona, Spain
    Zygote 11:347-54. 2003
    Our previous studies have shown that the addition of 100 mircroM cysteamine to the in vitro maturation (IVM) medium increased the embryo development of prepubertal goat oocytes...
  33. ncbi Understanding intestinal cysteamine bitartrate absorption
    Ranjan Dohil
    Department of Pediatrics, University of California, San Diego, La Jolla, California 92103 8450, USA
    J Pediatr 148:764-9. 2006
    To test the hypothesis that a controlled-release preparation of cysteamine, with fewer daily administrations, would improve the quality of life for patients with cystinosis.
  34. ncbi Influence of cysteamine supplementation and culture in portable dry-incubator on the in vitro maturation, fertilization and subsequent development of mouse oocytes
    Naiqing Chen
    Embryonics International, Gleneagles Hospital, Annexe Block, 01 38, 6A Napier Road, Singapore 258500, Singapore
    Theriogenology 63:2300-10. 2005
    ..In addition, the effects of cysteamine supplementation on maturation rate and embryonic development during in vitro maturation (IVM) and culture of ..
  35. ncbi The evaluation and treatment of gastrointestinal disease in children with cystinosis receiving cysteamine
    Ranjan Dohil
    Department of Pediatrics, University of California San Diego, UCSD Medical Center, Hillcrest, 200 West Arbor Drive, San Diego, CA 92103 8450, USA
    J Pediatr 143:224-30. 2003
    b>Cysteamine prevents organ damage in children with cystinosis, but may cause gastrointestinal (GI) symptoms. In this study we evaluated the nature of GI disease, and the value of omeprazole in controlling GI symptoms in these children.
  36. ncbi Cysteamine alters redox state, HIF-1alpha transcriptional interactions and reduces duodenal mucosal oxygenation: novel insight into the mechanisms of duodenal ulceration
    Tetyana Khomenko
    Pathology and Laboratory Medicine Service, Diagnostic and Molecular Medicine Health Care Group, Department of Medicine, VA Medical Center, CA 90822, USA
    Biochem Biophys Res Commun 317:121-7. 2004
    ..critical role of enhanced transcriptional activity of early growth response factor-1 (Egr-1) in early stages of cysteamine-induced duodenal ulcer in rats...
  37. pmc Pharmacokinetics of enteric-coated cysteamine bitartrate in healthy adults: a pilot study
    Jon A Gangoiti
    Department of Paediatrics, University of California, San Diego, La Jolla, California, USA
    Br J Clin Pharmacol 70:376-82. 2010
    b>Cysteamine bitartrate is taken lifelong, every 6 h and for the treatment of cystinosis. Recent studies using cysteamine for for other diseases such as neurodegenerative disorders adopt the same dosing regimen for cysteamine...
  38. ncbi Candida albicans infection delays duodenal ulcer healing in cysteamine-induced duodenal ulcers in rats
    Longxue Jin
    Department of Surgery, Keio University School of Medicine, Shinjuku ku, Tokyo, 160 8582, Japan
    Dig Dis Sci 53:2878-85. 2008
    ..albicans (the Candida group) or saline (the control group) was administered intragastrically into rats with a cysteamine-induced duodenal ulcer...
  39. pmc Mouse cumulus-denuded oocytes restore developmental capacity completely when matured with optimal supplementation of cysteamine, cystine, and cumulus cells
    Ping Zhou
    College of Animal Science and Veterinary Medicine, Shandong Agricultural University, Tai An City, People s Republic of China
    Biol Reprod 82:759-68. 2010
    Our objectives were to study how cysteamine, cystine, and cumulus cells (CCs), as well as oocytes interact to increase oocyte intracellular glutathione (GSH) and thereby to establish an efficient in vitro maturation system for cumulus-..
  40. ncbi The opposite effects of cysteamine on the acquisition of two different tasks in mice are associated with bidirectional testing-induced changes in hippocampal adenylyl cyclase activity
    J L Guillou
    Laboratoire de Neurosciences Comportementales et Cognitives, Universite de Bordeaux I, Talence, France
    Behav Neurosci 112:900-8. 1998
    ..Results showed that subcutaneous injections of cysteamine (160 mg/kg) (a) impaired acquisition of the spatial task while producing an opposite (i.e...
  41. ncbi HEC-cysteamine conjugates: influence of degree of thiolation on efflux pump inhibitory and permeation enhancing properties
    Deni Rahmat
    Department of Pharmaceutical Technology, Institute of Pharmacy, Leopold Franzens University of Innsbruck, Innrain 52, 6020 Innsbruck, Austria
    Int J Pharm 422:40-6. 2012
    Within the present study hydroxyethyl cellulose-cysteamine conjugates are investigated regarding biocompatibility, in situ gelling, permeation enhancing and efflux pump inhibitory properties...
  42. ncbi Gold nanoparticle probes: design and in vitro applications in cancer cell culture
    Gülcan Unak
    Ege University, Faculty of Science, Biochemistry Department, Bornova 35100, Izmir, Turkey
    Colloids Surf B Biointerfaces 90:217-26. 2012
    ..For the conjugation of (18)F-FDG and AuNPs, cysteamine was first bound to mannose triflate (Man-CA) before synthesizing of (18)F-FDG which has cysteamine sides ((18)..
  43. ncbi Cysteamine, glutathione and ionomycin treatments improve in vitro fertilization of prepubertal goat oocytes
    Aixa Urdaneta
    Departament de Ciencia Animal i dels Aliments, Facultat de Veterinaria, Universitat Autonoma de Barcelona, 08193 Bellaterra, Barcelona, Spain
    Zygote 12:277-84. 2004
    ..study was to improve in vitro embryo development of prepubertal goat oocytes by studying the effect of adding cysteamine to in vitro maturation medium, glutathione (GSH) to in vitro fertilization medium and ionomycin to the sperm ..
  44. ncbi Autophagy-mediated chemosensitization by cysteamine in cancer cells
    Xiao Mei Wan
    Hefei National Laboratory for Physical Sciences at Microscale and School of Life Sciences, University of Science and Technology of China, Hefei, Anhui 230027, People s Republic of China
    Int J Cancer 129:1087-95. 2011
    b>Cysteamine (CS) has many biomedical and clinical applications because of its excellent water solubility, low cytotoxicity and good biocompatibility. A previous study by Brawer et al...
  45. ncbi The interactions between cysteamine, cystine and cumulus cells increase the intracellular glutathione level and developmental capacity of goat cumulus-denuded oocytes
    Ping Zhou
    Laboratory for Animal Reproduction and Embryology, College of Animal Science and Veterinary Medicine, Shandong Agricultural University, Taian City 271018, Shandong Province, People s Republic of China
    Reproduction 135:605-11. 2008
    To improve in vitro maturation (IVM) of denuded oocytes (DOs), we observed the interactive effects of cysteamine, cystine and cumulus cells on the glutathione (L-gamma-glutamyl-L-cysteinyl-glycine; GSH) level and developmental capacity of ..
  46. ncbi Strict cysteamine dose regimen is required to prevent nocturnal cystine accumulation in cystinosis
    Elena N Levtchenko
    Department of Paediatric Nephrology, Radboud University Nijmegen Medical Centre, P O 9101, 6500 HB, Nijmegen, The Netherlands
    Pediatr Nephrol 21:110-3. 2006
    ..b>Cysteamine depletes intralysosomal cystine content, postpones the deterioration of renal function and the occurrence of ..
  47. pmc Steady-state pharmacokinetics and pharmacodynamics of cysteamine bitartrate in paediatric nephropathic cystinosis patients
    Eric B Belldina
    Department of Basic Pharmaceutical Sciences, West Virginia University, Morgantown, WV, USA
    Br J Clin Pharmacol 56:520-5. 2003
    b>Cysteamine is used to reduce tissue cystine content in patients suffering from nephropathic cystinosis...
  48. pmc Oral carnitine therapy in children with cystinosis and renal Fanconi syndrome
    W A Gahl
    Section of Human Biochemical Genetics, National Institute of Child Health and Human Development, Bethesda, Maryland 20892
    J Clin Invest 81:549-60. 1988
    ..However, its efficacy in restoring muscle carnitine to normal, and the optimal dosage regimen, have yet to be determined...
  49. ncbi Enteric-coated cysteamine for the treatment of paediatric non-alcoholic fatty liver disease
    R Dohil
    Department of Pediatrics, University of California, Rady Children s Hospital, San Diego, USA
    Aliment Pharmacol Ther 33:1036-44. 2011
    ..Hepatic fat accumulation and oxidative stress contribute to its pathogenesis. Cysteamine bitartrate readily traverses cellular membranes and is a potent antioxidant.
  50. ncbi Nephropathic cystinosis: posterior segment manifestations and effects of cysteamine therapy
    Ekaterini T Tsilou
    Ophthalmic Genetics and Visual Function Branch, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
    Ophthalmology 113:1002-9. 2006
    ..Treatment involves intracellular cystine depletion with oral cysteamine. A wide spectrum of ocular pathologic features has been associated with nephropathic cystinosis...
  51. ncbi Esomeprazole therapy for gastric acid hypersecretion in children with cystinosis
    Ranjan Dohil
    UCSD Medical Center, San Diego, CA 92103 8450, USA
    Pediatr Nephrol 20:1786-93. 2005
    Oral cysteamine therapy prevents natural disease progression in children with cystinosis, but it may cause severe gastrointestinal (GI) symptoms through gastric acid-hypersecretion...
  52. ncbi Enhanced ghrelin secretion in rats with cysteamine-induced duodenal ulcers
    Seiichiro Fukuhara
    Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku ku, Tokyo 160 8582, Japan
    Am J Physiol Gastrointest Liver Physiol 289:G138-45. 2005
    ..b>Cysteamine inhibits the release of somatostatin and induces the formation of duodenal ulcers in rats...
  53. ncbi Swallowing dysfunction in 101 patients with nephropathic cystinosis: benefit of long-term cysteamine therapy
    Barbara C Sonies
    Oral Motor Function Section, Physical Disabilities Branch, Rehabilitation Medicine Department, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, MD 20892, USA
    Medicine (Baltimore) 84:137-46. 2005
    ..function) increased (that is, worsened) with the number of years that a patient was not receiving treatment with cysteamine, the cystine-depleting agent of choice in cystinosis...
  54. ncbi Evaluation of treatment with cysteamine eyedrops for cystinosis with confocal microscopy
    Rui Tavares
    Ophthalmology Department, Coimbra University Hospital, Coimbra, Portugal
    Cornea 28:938-40. 2009
    The purpose of this study was to evaluate the efficacy of cysteamine eyedrops 0...
  55. ncbi In vitro maturation and glutathione synthesis of porcine oocytes in the presence or absence of cysteamine under different oxygen tensions: role of cumulus cells
    Y Z Bing
    National Agricultural Research Center for Tohoku Region, Morioka, Iwate, Japan
    Reprod Fertil Dev 14:125-31. 2002
    ..vitro maturation (IVM) and glutathione (GSH) synthesis of porcine oocytes cultured in the presence or absence of cysteamine under different oxygen tensions, and on their subsequent male pronucleus formation after in vitro fertilization (..
  56. ncbi Pantetheinase activity of membrane-bound Vanin-1: lack of free cysteamine in tissues of Vanin-1 deficient mice
    G Pitari
    Dipartmento di Biologia di Base ed Applicata Università di L Aquila, Italy
    FEBS Lett 483:149-54. 2000
    ..is an ubiquitous enzyme which in vitro has been shown to recycle pantothenic acid (vitamin B5) and to produce cysteamine, a potent anti-oxidant...
  57. ncbi Nephropathic cystinosis: late complications of a multisystemic disease
    Galina Nesterova
    Section on Human Biochemical Genetics, Human Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892 1851, USA
    Pediatr Nephrol 23:863-78. 2008
    ..Prior to renal transplantation and cystine-depleting therapy with cysteamine for children with nephropathic cystinosis, their lifespan was approximately 10 years...
  58. ncbi The influence of trehalose, taurine, cysteamine and hyaluronan on ram semen Microscopic and oxidative stress parameters after freeze-thawing process
    Mustafa Numan Bucak
    Ankara University, Veterinary Faculty, Department of Reproduction and Artificial Insemination, 06610 Ankara, Turkey
    Theriogenology 67:1060-7. 2007
    ..diluted with a Tris-based extender containing additives including trehalose (50, 100mM), taurine (25, 50mM), cysteamine (5, 10mM), and hyaluronan (0...
  59. ncbi Effect of cysteamine on oxidative stress-induced cell death of human corneal endothelial cells
    Young Joo Shin
    Department of Ophthalmology, Hallym University College of Medicine, Seoul, Republic of Korea
    Curr Eye Res 36:910-7. 2011
    The principal objective of this study was to evaluate the protective effect of cysteamine against the oxidative stress-induced cell death of human corneal endothelial cells.
  60. ncbi Cystinosis
    William A Gahl
    Heritable Disorders Branch, National Institute of Child Health and Human Development, Bethesda, MD 20892 1830, USA
    N Engl J Med 347:111-21. 2002
  61. ncbi Evolution of ocular manifestations in nephropathic cystinosis: a long-term study of a population treated with cysteamine
    Pascal Dureau
    Service d Ophtalmologie, Hopital Necker Enfants Malades, Paris, France
    J Pediatr Ophthalmol Strabismus 40:142-6. 2003
    ..Renal transplantation and oral cysteamine have improved the general prognosis of the disease, and ocular manifestations are now the most common ..
  62. pmc A multicentre randomised double masked clinical trial of a new formulation of topical cysteamine for the treatment of corneal cystine crystals in cystinosis
    E T Tsilou
    Ophthalmic Genetics and Visual Function Branch, National Eye Institute, National Institutes of Health, Bethesda, MD 20892, USA
    Br J Ophthalmol 87:28-31. 2003
    To evaluate the safety and efficacy of a new topical cysteamine formulation, stable at room temperature, for the treatment of corneal cystine crystals in cystinosis.
  63. ncbi Corneal crystals in nephropathic cystinosis: natural history and treatment with cysteamine eyedrops
    W A Gahl
    Section on Human Biochemical Genetics, Heritable Disorders Branch, National Institute of Child Health and Human Development, Bethesda, Maryland 20892, USA
    Mol Genet Metab 71:100-20. 2000
    ..Administration of 0.55% cysteamine eyedrops, given 6 to 12 times per day, dissolved corneal cystine crystals in 10 representative patients with ..
  64. ncbi Gel formulations for treatment of the ophthalmic complications in cystinosis
    Barbara Buchan
    School of Pharmacy and Life Sciences, The Robert Gordon University, Schoolhill, Aberdeen, UK
    Int J Pharm 392:192-7. 2010
    ..It is treated by regular administration of the aminothiol, cysteamine. Corneal crystal deposition is one of the most troublesome complications affecting patients and requires the ..
  65. ncbi Development of Fanconi syndrome during infancy in a patient with cystinosis
    Elena Levtchenko
    Acta Paediatr 95:379-80. 2006
  66. ncbi Nephropathic cystinosis in adults: natural history and effects of oral cysteamine therapy
    William A Gahl
    National Human Genome Research Institute and Intramural Office of Rare Diseases, National Institutes of Health, Bethesda, Maryland 20892 1851, USA
    Ann Intern Med 147:242-50. 2007
    The full burden of nephropathic cystinosis in adulthood and the effects of long-term oral cysteamine therapy on its nonrenal complications have not been elucidated.
  67. ncbi Functionalization of gold cysteamine self-assembled monolayer with ethylenediaminetetraacetic acid as a novel nanosensor
    Reza Karimi Shervedani
    Department of Chemistry, University of Isfahan, Isfahan 81746 73441, Islamic Republic of Iran
    Anal Chim Acta 587:254-62. 2007
    Electrochemical characterization of gold cysteamine self-assembled monolayer, in situ functionalized with ethylenediaminetetraacetic acid (Au-CA-EDTA SAM), is described by cyclic voltammetry (CV), electrochemical impedance spectroscopy (..
  68. ncbi Growth retardation in children with cystinosis
    M Besouw
    Department of Pediatric Nephrology, University Hospital Gasthuisberg, Leuven, Belgium
    Minerva Pediatr 62:307-14. 2010
    ..The cystine depleting drug cysteamine slows down the progression of renal disease, protects extra-renal organs, accelerates growth and therefore ..
  69. ncbi Fertility status in male cystinosis patients treated with cysteamine
    Martine T P Besouw
    Department of Pediatrics Pediatric Nephrology, University Hospital Gasthuisberg, Leuven, Belgium
    Fertil Steril 93:1880-3. 2010
    To analyze the fertility status in adult, male cystinosis patients treated with cysteamine. Cystinosis is an autosomal recessive disease leading to intralysosomal cystine accumulation...
  70. ncbi Twice-daily cysteamine bitartrate therapy for children with cystinosis
    Ranjan Dohil
    Department of Pediatrics, University of California San Diego, La Jolla, CA 92103 8450, USA
    J Pediatr 156:71-75.e1-3. 2010
    Cystinosis causes renal and other organ failure. Regular 6-hourly cysteamine bitartrate (Cystagon; Mylan, Morgantown, West Virginia) reduces intracellular cystine and the rate of organ deterioration...
  71. ncbi Studies on the anti-inflammatory and anti-nociceptive effects of melatonin in the rat
    Siham M el-Shenawy
    Department of Pharmacology, National Research Centre, Tahrir St, Dokki, Cairo, Egypt
    Pharmacol Res 46:235-43. 2002
    ..5 mg kg(-1). Melatonin enhanced the anti-inflammatory effect of cysteamine (300 mg kg(-1), s.c.) in the carrageenan-induced paw oedema...
  72. ncbi A comparison of the effectiveness of cysteamine and phosphocysteamine in elevating plasma cysteamine concentration and decreasing leukocyte free cystine in nephropathic cystinosis
    L A Smolin
    Department of Pediatrics, School of Medicine, University of California, San Diego, La Jolla 92093
    Pediatr Res 23:616-20. 1988
    b>Cysteamine (beta-mercaptoethylamine, MEA) is currently used to treat children with nephropathic cystinosis...
  73. pmc A novel approach to enhancing cellular glutathione levels
    Pamela Maher
    The Salk Institute, La Jolla, California 92037, USA
    J Neurochem 107:690-700. 2008
    ..In this study, we characterize an additional EC conjugate, cysteamine-EC, that is 5- to 10-fold more potent than the earlier conjugates...
  74. ncbi Comparison of cystine determination in mixed leukocytes vs polymorphonuclear leukocytes for diagnosis of cystinosis and monitoring of cysteamine therapy
    Elena Levtchenko
    Department of Paediatric Nephrology, University Medical Centre St Radboud, Nijmegen, The Netherlands
    Clin Chem 50:1686-8. 2004
  75. ncbi Cysteamine supplementation of in vitro maturation media: a review
    S Deleuze
    Université de Liège Clinique des animaux de compagnie et des équidés, Liege, Belgium
    Reprod Domest Anim 45:e476-82. 2010
    ..Addition of low molecular weight compounds to culture media, such as cysteamine, can increase GSH levels by increasing cysteine uptake...
  76. ncbi Potential antidepressant properties of cysteamine on hippocampal BDNF levels and behavioral despair in mice
    Chu Hsin Shieh
    Institute of Anatomy and Cell Biology, National Yang Ming University, Taipei, Taiwan
    Prog Neuropsychopharmacol Biol Psychiatry 32:1590-4. 2008
    ..Recent work has found that cysteamine and its related agent, cystamine, are neuroprotective in Huntington's disease mice, and act by enhancing the ..
  77. ncbi The origin of halitosis in cystinotic patients due to cysteamine treatment
    Martine Besouw
    Department of Pediatric Nephrology, Radboud University Nijmegen Medical Centre, Nijmegen, P O Box 9101, 6500 HB Nijmegen, The Netherlands
    Mol Genet Metab 91:228-33. 2007
    Cystinosis is a rare autosomal recessive disorder characterized by the intralysosomal accumulation of cystine. Cysteamine removes cystine from the lysosome and slows down the progression of the disease...
  78. ncbi Early oral cysteamine therapy for nephropathic cystinosis
    William A Gahl
    Section on Human Biochemical Genetics, National Institute of Child Health and Human Development, National Institutes of Health, MSC 1851 Building 10, Room 10C 103, 10 Center Drive, Bethesda, Maryland 20892 1851, USA
    Eur J Pediatr 162:S38-41. 2003
    ..The mainstay of cystinosis therapy is oral cysteamine (Cystagon)...
  79. ncbi Developmental toxicity of cysteamine in the rat: effects on embryo-fetal development
    D A Beckman
    Division of Developmental Biology, Nemours Research Programs, Alfred I duPont Hospital for Children, Wilmington, Delaware 19899, USA
    Teratology 58:96-102. 1998
    The reproductive and developmental safety of cysteamine has become an important issue to children with cystinosis because renal transplants and treatment with cysteamine reduce the complications associated with cystinosis and increase the ..
  80. ncbi Cysteamine supplementation of in vitro maturation medium, in vitro culture medium or both media promotes in vitro development of buffalo (Bubalus bubalis) embryos
    T Anand
    Animal Biotechnology Centre, National Dairy Research Institute, Karnal 132 001, India
    Reprod Fertil Dev 20:253-7. 2008
    ..effects of supplementation of in vitro maturation (IVM) or in vitro culture (IVC) or both IVM and IVC media with cysteamine on the yield, hatching rate (HR) and total cell number (TCN) of buffalo blastocysts were examined...
  81. ncbi Effects of cysteamine on oxidative status in cerebral cortex of rats
    Adriana Kessler
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos 2600, CEP 90 035 003, Porto Alegre, Rio Grande do Sul, Brazil
    Metab Brain Dis 23:81-93. 2008
    ..Tissue damage depends on cystine accumulation, but the mechanisms of this damage are still obscure. Cysteamine administration depletes cystine accumulated, increasing survive of affected patients...
  82. ncbi Cystamine and cysteamine prevent 3-NP-induced mitochondrial depolarization of Huntington's disease knock-in striatal cells
    Zhengkuan Mao
    Department of Psychiatry, University of Alabama at Birmingham, Birmingham, AL 35294 0017, USA
    Eur J Neurosci 23:1701-10. 2006
    ..of Huntington's disease (HD); however, the precise mechanism(s) by which cystamine and the related compound cysteamine are beneficial remain to be elucidated...
  83. ncbi Thiolated polymers: synthesis and in vitro evaluation of polymer-cysteamine conjugates
    A Bernkop-Schnürch
    Center of Pharmacy, Institute of Pharmaceutical Technology and Biopharmaceutics, University of Vienna, Althanstrasse 14, A 1090 Vienna, Austria
    Int J Pharm 226:185-94. 2001
    ..Mediated by a carbodiimide cysteamine was covalently linked to sodium carboxymethylcellulose (CMC) and polycarbophil (PCP)...
  84. ncbi CYTE-I-HD: phase I dose finding and tolerability study of cysteamine (Cystagon) in Huntington's disease
    Richard Dubinsky
    Department of Neurology, University of Kansas Medical Center, Kansas City, 66160, USA
    Mov Disord 21:530-3. 2006
    ..b>Cysteamine, the dimer of cystamine, is an orphan drug approved for the treatment of nephropathic cystinosis and has a ..
  85. pmc Role of iron in the pathogenesis of cysteamine-induced duodenal ulceration in rats
    Tetyana Khomenko
    Diagnostic and Molecular Medicine Health Care Group, VA Medical Ctr, 5901 East 7St, Long Beach, CA 90822 5201, USA
    Am J Physiol Gastrointest Liver Physiol 296:G1277-86. 2009
    b>Cysteamine induces perforating duodenal ulcers in rats within 24-48 h. This reducing aminothiol generates hydrogen peroxide in the presence of transition metals (e.g...
  86. ncbi Effect of cysteamine on redox-sensitive thiol-containing proteins in the duodenal mucosa
    Tetyana Khomenko
    Pathology and Laboratory Medicine Service, Diagnostic and Molecular Medicine Health Care Group, VA Medical Center, Long Beach, CA 90822, USA
    Biochem Biophys Res Commun 309:910-6. 2003
    Recent studies from our laboratory demonstrated that Egr-1 is upregulated in the rat duodenal mucosa during cysteamine-induced duodenal ulceration and that antisense egr-1 oligonucleotide aggravates the duodenal ulcers...
  87. ncbi Influence of cysteamine on in vitro maturation, in vitro and in vivo fertilization of equine oocytes
    S Deleuze
    Faculte de Medecine Veterinaire, Département des Sciences Cliniques Clinique Equine, Universite de Liege, Liege, Belgium
    Reprod Domest Anim 45:1-7. 2010
    Contents The effect of cysteamine on in vitro nuclear and cytoplasmic maturation of equine oocytes collected by transvaginal ultrasound guided follicular aspiration was assessed...
  88. ncbi (S)-(2-(2'-Pyridyl)ethyl)cysteamine and (S)-(2-(2'-pyridyl)ethyl)-d,l-homocysteine as ligands for the "fac-[M(CO)(3)](+)" (M = Re, (99m)Tc) core
    O Karagiorgou
    Institutes of Radioisotopes Radiodiagnostic Products, Biology, and Materials Science, NCSR Demokritos, Ag Paraskevi, Athens, Greece
    Inorg Chem 44:4118-20. 2005
    The reaction of fac-[NEt(4)](2)[Re(CO)(3)Br(3)] with (S)-(2-(2'-pyridyl)ethyl)cysteamine, L(1), in methanol leads to the formation of the cationic fac-[Re(CO)(3)(NSN)][Br] complex, 1, with coordination of the nitrogen of the pyridine, the ..
  89. pmc The killing effect of 4-S-cysteaminylphenol, a newly synthesised melanin precursor, on B16 melanoma cell lines
    I Yamada
    Department of Radiology, School of Medicine, Tokyo Medical and Dental University, Japan
    Br J Cancer 63:187-90. 1991
    ..Thus, 4-S-CAP appears to provide a new, effective cytotoxic agent for rational chemotherapy of malignant melanomas...
  90. pmc Pharmacokinetics of cysteamine bitartrate following gastrointestinal infusion
    Meredith C Fidler
    Department of Paediatrics and Family, University of California at San Diego, La Jolla, California, USA
    Br J Clin Pharmacol 63:36-40. 2007
    Although cysteamine was first used in the treatment of cystinosis in 1976 and approved by the FDA as cysteamine bitartrate (Cystagon) in 1994, surprisingly little pharmacological data are available for this compound...
  91. ncbi Phase advances of circadian rhythms in somatostatin depleted rats: effects of cysteamine on rhythms of locomotor activity and electrical discharge of the suprachiasmatic nucleus
    C Fukuhara
    Laboratory of Integrative Brain Functions, Mitsubishi Kasei Institute of Life Sciences, Tokyo, Japan
    J Comp Physiol A 175:677-85. 1994
    ..Intraperitoneal administration of cysteamine (200 mg/kg), a somatostatin depletor, significantly reduced somatostatin level in the in vivo SCN 5 min after ..
  92. ncbi [From gene to disease: cystinosis]
    E N Levtchenko
    afd Kindergeneeskunde, Universitair Medisch Centrum St Radboud, Postbus 9101, 6500 HB Nijmegen
    Ned Tijdschr Geneeskd 148:476-8. 2004
    ..The amino-thiol cysteamine depletes lysosomal cystine content by a disulfide-exchange reaction with cystine...
  93. ncbi Exogenous gamma-glutamyl cycle compounds supplemented to in vitro maturation medium influence in vitro fertilization, culture, and viability parameters of porcine oocytes and embryos
    Brian D Whitaker
    Department of Animal and Poultry Sciences, Virginia Polytechnic Institute and State University, Blacksburg, VA 24060 0306, USA
    Theriogenology 62:311-22. 2004
    ..Porcine oocytes were matured in NCSU 23 medium supplemented with either l-cysteine (3.3 mM), l-cysteamine (150 P < 0.05microM), l-cysteine and l-cystemaine, l-glycine (1, 2.5, or 5 mM), l-glutamate (1, 2...
  94. ncbi Treatment of cystinosis: simple in principle, difficult in practice
    Jerry A Schneider
    J Pediatr 145:436-8. 2004
  95. ncbi Hematological manifestations of nephropathic cystinosis
    Ashkan Emadi
    Department of Oncology, Sidney Kimmel Comprehensive Cancer Center, Baltimore, MD 21231 1000, USA
    Acta Haematol 119:169-72. 2008
    ....
  96. ncbi Noncirrhotic portal hypertension in association with juvenile nephropathic cystinosis: case presentation and review of the literature
    P DiDomenico
    Department of Pediatric Nephrology, Lucile Packard Children s Hospital, Stanford University Medical Center, Palo Alto, California, USA
    J Inherit Metab Dis 27:693-9. 2004
    ..The patient had a history of poor compliance with her prescribed cysteamine therapy. Routine examination revealed normal liver function without hepatomegaly but asymptomatic splenomegaly...
  97. ncbi [Cystinosis: an infrequent cause of distal myopathy]
    F J Rodríguez-Gómez
    Servicio de Medicina Interna, Hospital Juan Ramon Jimenez, 21005 Huelva
    Rev Neurol 40:156-8. 2005
    ..Cystinosis is a hereditary disease with clinical symptoms that are caused by the accumulation of cystine crystals in different tissues. Distal vacuolar myopathy has been reported as one of its later complications...
  98. ncbi Cysteamine prevents and reverses the inhibition of creatine kinase activity caused by cystine in rat brain cortex
    Rochele M Müller Fleck
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos 2600 anexo, CEP 90 035 003 Porto Alegre, RS, Brazil
    Neurochem Int 46:391-7. 2005
    ..kinetic studies and investigated the effects of GSH, a biologically occurring thiol group protector, and cysteamine, the drug used for cystinosis treatment, to better understand the effect of cystine on creatine kinase activity...
  99. pmc Growth hormone producing prolactinoma in juvenile cystinosis: a simple coincidence?
    Martine T P Besouw
    Department of Pediatric Nephrology, University Medical Centre St Radboud, Nijmegen, The Netherlands
    Pediatr Nephrol 23:307-10. 2008
    ..Treatment with oral cysteamine and topical cysteamine eye drops was started...
  100. ncbi Promotion of oxidative stress in kidney of rats loaded with cystine dimethyl ester
    Virginia Cielo Rech
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, Universidade, Rua Ramiro Barcelos 2600, CEP 90 035 003, Porto Alegre, RS, Brazil
    Pediatr Nephrol 22:1121-8. 2007
    ..Animals were injected twice a day with 1.6 micromol/g body weight CDME and/or 0.26 micromol/g body weight cysteamine (CSH) from the 16th to the 20th postpartum day and killed after 1 or 12 h...
  101. ncbi Effects on in vitro embryo development and intracellular glutathione content of the presence of thiol compounds during maturation of prepubertal goat oocytes
    Elisabet Rodríguez-González
    Departament de Ciencia Animal i dels Aliments, Facultat de Veterinaria, Universitat Autonoma de Barcelona, Bellaterra, Barcelona, Spain
    Mol Reprod Dev 65:446-53. 2003
    ..The present study was carried out to determine whether supplementation of the IVM medium with 100 microM of cysteamine, 100 microM of beta-mercaptoethanol, 0.57 mM of cysteine, and 0...

Research Grants29

  1. Lentiviral-transduced hematopoictic stem cell transplantation for cystinosis
    Stephanie Cherqui; Fiscal Year: 2013
    ..Cystine accumulation eventually leads to multi-organ dysfunction. The drug cysteamine reduces the intracellular concentration of cystine...
  2. Integrative Genomics of Vanin Gene Expression in Relation to CVD Risk
    Eric Moses; Fiscal Year: 2013
    ..is a pantetheinase that catalyzes the hydrolysis of D-pantetheine generating pantothenate (vitamin B5) and cysteamine, a potent anti-oxidant known to prevent lipid peroxidation...
  3. NUTRITIONAL REGULATION OF CYSTEINE DIOXYGENASE
    Martha H Stipanuk; Fiscal Year: 2013
    ..These two unique enzymes are cysteine dioxygenase (CDO), encoded by CDO1, and cysteamine dioxygenase (ADO, 2-aminoethanethiol dioxygenase), which we recently showed to be encoded by human gene C10orf22...
  4. Kidney-targeted gene delivery for cystinosis
    Stephanie Cherqui; Fiscal Year: 2012
    ..Cystine accumulation eventually leads to multi-organ dysfunction. The drug cysteamine reduces the intracellular concentration of cystine...
  5. Toxicology studies for gene-modified stem cell transplantation for cystinosis
    Stephanie Cherqui; Fiscal Year: 2013
    ..Cystine accumulation leads to multi-organ dysfunction. The drug cysteamine reduces the intracellular cystine content...
  6. Development of Cysteamine as a Medical Radiation Countermeasure
    John L Zenk; Fiscal Year: 2010
    ..Humanetics is working to develop the small stable molecule BIO 600 as a radioprotective countermeasure to protect people from death due to the lethal effects of ARS. ..
  7. Molecular Basis of Infantile NCL
    Sandra L Hofmann; Fiscal Year: 2010
    ..We will investigate the mechanism of action of cysteamine, the first rationally designed drug introduced for the treatment of INCL, through biochemical and metabolic ..
  8. Molecular Mechanisms in Arterial Calcification
    Robert A Terkeltaub; Fiscal Year: 2012
    ..precursor cells linked with both PPi deficiency and driven by increased vanin-1 pantetheinase activity, cysteamine generation, and consequent GSH depletion...
  9. LYSOSOMAL TRANSPORT OF NUCLEOSIDES AND NUCLEOBASES
    RONALD PISONI; Fiscal Year: 1992
    ..Lysosomal amino acid studies have aided our understanding of the basis of cysteamine treatment of cystinosis...
  10. SOMATOSTATIN-28 CONTROL OF VASOPESSIN & EPINEPHRINE
    MARVIN BROWN; Fiscal Year: 1992
    ..Depletion of brain somatostatin with cysteamine attenuates hemorrhage-induced elevation of plasma concentration of vasopressin...
  11. ANTICYSTINOTIC AGENTS, RESEARCH AND DEVELOPMENT
    MAXIMILLIAN VON STRANDTMANN; Fiscal Year: 1992
    ..Cystinosis is presently controlled by administration of cysteamine or phosphocysteamine. The repulsive odor of cysteamine frequently causes outright rejection by the patient...
  12. MAMMALIAN CYSTEINE METABOLISM
    Owen Griffith; Fiscal Year: 1993
    ..inhibitors of the phosphopantetheine synthesis will be made and used to control taurine formation via the cysteamine pathway...
  13. DEVELOPMENT OF SOMATOSTATIN-CONTAINING NEURONS
    Donna Ferriero; Fiscal Year: 1991
    ..b>Cysteamine, which reversibly depletes somatostatin immunoreactivity and biologic activity in the retina and central nervous ..
  14. MECHANISMS OF RADIOPROTECTION & TOXICITY OF SH COMPOUNDS
    KATHRYN HELD; Fiscal Year: 1992
    ..The thiols to be used are dithiothreitol, glutathione, and cysteamine and its derivatives, including WR-1065...
  15. Nutritional Regulation of y-Glutamylcysteine Synthetase
    MARTHA STIPANUK; Fiscal Year: 2007
    ..b>cysteamine) to downregulate GCS subunit expression and/or GCS activity; (7) to determine if expression of GCS subunit genes ..
  16. HIV Neuroprotection Workshop
    Joseph Berger; Fiscal Year: 2004
    ..The proposed conference is the first of its kind to highlight neuroprotection in HIV CNS and PNS disorders. By coupling this meeting with the International Symposium on Neurovirology this synergy can be exploited. ..
  17. NEUROSCIENCE OF HIV CONFERENCE
    Joseph Berger; Fiscal Year: 2002
    ..Dr. Brian Wigdahl, international co-chair of the ISNV, is a co-investigator on this grant. ..
  18. GENETICS OF AGED RELATED CATARACTS IN SALISBURY MARYLAND
    Nathan Congdon; Fiscal Year: 2002
    ....
  19. JCV Virus Expression in Oropharynx and HIV
    Joseph Berger; Fiscal Year: 2004
    ..This line of investigation would add credence to an oropharyngeal mechanism of JCV transmission and may ultimately lead to the clinical identification of an acute illness attributable to this pathogen. ..
  20. Intestinal colonization by C.albicans and enteric bacteria
    Carol Kumamoto; Fiscal Year: 2008
    ..unreadable] [unreadable] [unreadable]..
  21. Regulation of Drug Resistance Genes in C. Albicans
    Carol Kumamoto; Fiscal Year: 2007
    ..The long-term goal of these studies is to contribute to a more informed use of fluconazole with respect to prophylaxis, drug dosage regimens and the development of fluconazole resistant strains. [unreadable] [unreadable] [unreadable]..
  22. PKC Cys switches -- novel cancer prevention targets
    CATHERINE O BRIAN; Fiscal Year: 2006
    ....
  23. Regulation of Fungal Invasive Growth
    Carol Kumamoto; Fiscal Year: 2006
    ..cerevisiae. Experiments are proposed to identify interactors that bind to the gene products of interest and to elucidate the pathways in which these gene products participate. ..
  24. MR PREDICTORS OF RESPONSE TO HAART IN HIV DEMENTIA
    Joseph Berger; Fiscal Year: 2004
    ..It will also open novel avenues for exploring the differential sensitivities of these pathways to HAART, and suggest unique therapeutic options. ..
  25. Novel therapeutics for chronic pain
    Paul Wood; Fiscal Year: 2005
    ....
  26. Structural analysis of TB-RBP, a DNA/RNA-binding protein
    Jon Robertus; Fiscal Year: 2005
    ..Together, this work should elucidate the molecular structure and the mode of action and regulation of an important new class of DNA/RNA-binding proteins of great interest to the scientific and medical community. ..