Zobrazeno 1 - 10
of 13
pro vyhledávání: '"Jean Deb Butler"'
Autor:
Sondra W. Levin, Zhongjian Zhang, Anil B. Mukherjee, Susan Sklower Brooks, Krystyna E. Wisniewski, Jean Deb Butler
Publikováno v:
Nature medicine. 7(4)
Neuronal ceroid lipofuscinoses (NCLs) are the most common hereditary neurodegenerative diseases of childhood. The infantile form, INCL, is caused by lysosomal palmitoyl-protein thioesterase (PPT) deficiency, which impairs the cleavage of thioester li
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::94906172734052d8e2a52863ba71b3e6
https://pubmed.ncbi.nlm.nih.gov/11283676
https://pubmed.ncbi.nlm.nih.gov/11283676
Autor:
Rodney E. Ulane, Van S. Hubbard, Marta M. Ulane, Alessandro Peri, Jean Deb Butler, Lucio Miele
Publikováno v:
Clinica chimica acta; international journal of clinical chemistry. 230(2)
The cystic fibrosis (CF) gene defect may be associated with a defect in membrane recycling. We have investigated the metabolism of the main constituent of plasma membrane, phosphatidylcholine (PC). In this study of platelets and fibroblasts, we show
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::0daf6eb6f3601ffaa30641c488181e88
https://pubmed.ncbi.nlm.nih.gov/7834862
https://pubmed.ncbi.nlm.nih.gov/7834862
Autor:
Virginia K. Weise, Jean Deb Butler, Jeanne G. Waggoner, Harvey R. Gralnick, Dawn G. Goodman, Bennett L. Blitzer, Paul F. Teychenne, David W. Towne, E. Anthony Jones, Ian B. Walters, Irwin J. Kopin, Paul D. Berk
Publikováno v:
Gastroenterology. 74:664-671
A highly reproducible model of fulminant hepatic failure was developed by administering intravenously the selective hepatotoxin galactosamine hydrochloride (4.25 mmoles per kg) to genetically uniform rabbits. The great majority of rabbits died betwee
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::5110af748606fafdec12abd5af97e619
https://doi.org/10.1016/0016-5085(78)90241-x
https://doi.org/10.1016/0016-5085(78)90241-x
Publikováno v:
Pediatric Research. 15:1063-1067
Cysteamine is the most effective agent known for the reduction of the elevated cystine content of cells from patients with cystinosis. A defect in endogenous cysteamine generation could account for many of the metabolic features of this disorder. To
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::962aef140bd9759145a05a68a3437a30
https://doi.org/10.1203/00006450-198107000-00018
https://doi.org/10.1203/00006450-198107000-00018
Publikováno v:
Biochemical and Biophysical Research Communications. 89:504-511
Glutathione synthetase deficiency results in decreased cellular glutathione content and consequent overproduction of 5-oxoproline. L-serine in borate buffer inhibits γ-glutamyl transpeptidase, the major catabolic enzyme for glutathione. Treatment of
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5d4879a33f5d8188aa91af61bc69a7eb
https://doi.org/10.1016/0006-291x(79)90658-2
https://doi.org/10.1016/0006-291x(79)90658-2
Autor:
Martin Zatz, Jean DeB. Butler
Publikováno v:
The Journal of pediatrics. 102(5)
CYSTINOSIS is an autosomal recessive genetic disease in which cystine characteristically accumulates in the lysosomes of cells. The organ most affected is the kidney; the Fanconi syndrome develops early in life, and is followed by progressive renal f
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3b7f4223f54a25c54887e106e57637ba
https://pubmed.ncbi.nlm.nih.gov/6842344
https://pubmed.ncbi.nlm.nih.gov/6842344
Publikováno v:
In vitro. 16(8)
Extracellular cysteine concentrations between 0.5 and 2.5 mM resulted in death of normal but not cystinotic cells grown in Eagle's minimal essential medium containing supplemental fetal bovine serum and antibiotics. Differential cell survival was det
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::509f4ddefeeaf28067d480c9a0643c7d
https://pubmed.ncbi.nlm.nih.gov/7419235
https://pubmed.ncbi.nlm.nih.gov/7419235
Publikováno v:
Life sciences. 38(20)
Although progesterone is known to produce quiescence in the mammalian uterus, the mechanism of this effect is not clearly understood. Here, we report that uteroglobin, a progesterone-induced small molecular weight (16K) protein, inhibits phospholipas
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b08b348e8669181662fcb10ff616ad3a
https://pubmed.ncbi.nlm.nih.gov/3084897
https://pubmed.ncbi.nlm.nih.gov/3084897
Autor:
Wayne K. Hoffman, Jean Deb Butler, Sheldon Orloff, Peter A. Lalley, Joseph D. Schulman, Anil B. Mukherjee
Publikováno v:
In vitro. 17(8)
Using lipochromosomes (phospholipid-entrapped chromosomes) were have transferred the human HGPRT gene into HGPRT deficient mouse cells (A9) with a frequency of approximately 1 x 19(-5) (Mukherjee et al. Proc. Natl. Acad. Sci. USA 75: 1361-1365; 1978)
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a7533e7e313d0a18804a175912a89dac
https://pubmed.ncbi.nlm.nih.gov/7199026
https://pubmed.ncbi.nlm.nih.gov/7199026
Autor:
Uwe K. Schumacher, Kuzhiyilethu K. Kumaroo, Jerome A. Donlon, McDonald K. Horne, Ramanathapuram Manjunath, Ryojiro Fujita, Sondra W. Levin, Jean Deb Butler, Anil B. Mukherjee
Publikováno v:
Biochemical pharmacology. 36(5)
Uteroglobin, a steroid-dependent, small molecular weight (15K) protein in the rabbit, inhibited thrombin-induced aggregation of both rabbit and human gel-filtered platelets (GFP). GFP aggregation by arachidonic acid was not affected by uteroglobin. T
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::25fbd9e99ddabdc7b75693e802ea9c71
https://pubmed.ncbi.nlm.nih.gov/3827955
https://pubmed.ncbi.nlm.nih.gov/3827955