Zobrazeno 1 - 10
of 26
pro vyhledávání: '"Adele Cooney"'
Publikováno v:
Neuroscience Letters. 590:134-137
In Parkinson’s disease (PD) alpha-synuclein oligomers are thought to be pathogenic, and 3,4-dihydroxyphenylacetaldehyde (DOPAL), an obligate aldehyde intermediate in neuronal dopamine metabolism, potently oligomerizes alpha-synuclein. PD involves a
Autor:
Adele Cooney, Yunden Jinsmaa, Yehonatan Sharabi, David S. Goldstein, Irwin J. Kopin, Patti Sullivan
Publikováno v:
Journal of Neurochemistry. 133:14-25
Repeated systemic administration of the mitochondrial complex I inhibitor rotenone produces a rodent model of Parkinson's disease (PD). Mechanisms of relatively selective rotenone-induced damage to nigrostriatal dopaminergic neurons remain incomplete
Autor:
Yunden Jinsmaa, Yehonatan Sharabi, Courtney Holmes, Rachel Sullivan, Irwin J. Kopin, Daniel J. Gross, Patti Sullivan, David S. Goldstein, Adele Cooney
Publikováno v:
Journal of Neurochemistry. 123:932-943
Parkinson's disease entails profound loss of nigrostriatal dopaminergic terminals, decreased vesicular uptake of intraneuronal catecholamines, and relatively increased putamen tissue concentrations of the toxic dopamine metabolite, 3,4-dihydroxypheny
Autor:
Patti Sullivan, Adele Cooney, John E. Casida, Yunden Jinsmaa, David S. Goldstein, Breanna Ford
Publikováno v:
Chemical Research in Toxicology
The dopamine metabolite 3,4-dihydroxyphenylacetaldehyde (DOPAL) is detoxified mainly by aldehyde dehydrogenase (ALDH). We find that the fungicide benomyl potently and rapidly inhibits ALDH and builds up DOPAL in vivo in mouse striatum and in vitro in
Autor:
Jane M. Quirk, Sang H. Shin, Gary J. Murray, Raphael Schiffmann, Roscoe O. Brady, Christine R. Kaneski, Adele Cooney, Stefanie Kluepfel-Stahl
Publikováno v:
Pharmacogenetics and Genomics. 18:773-780
To examine the relationship between types and locations of mutations of the enzyme alpha-galactosidase (Gal) A in Fabry disease and the response to the pharmacological chaperone 1-deoxygalactonojirimycin (DGJ).T cells grown from normal individuals or
Autor:
Roscoe O. Brady, Jane M. Quirk, Adele Cooney, Raphael Schiffmann, Christine R. Kaneski, Sang-Hoon Shin, Gary J. Murray, Stefanie Kluepfel-Stahl
Publikováno v:
Biochemical and Biophysical Research Communications. 359:168-173
As a prerequisite for clinical trials of pharmacological chaperone therapy (PCT) for Fabry disease, we developed a rapid screening assay for enhancement of endogenous alpha-galactosidase A (alpha-Gal A) in patient-derived cells. We used a T-cell base
Autor:
Silvia Santamarina-Fojo, Nancy K. Dwyer, Christian A. Combs, Catherine L. Knapper, Joan Blanchette-Mackie, Stephen J. Demosky, Edward B. Neufeld, H. Bryan Brewer, John A. Stonik, Adele Cooney, Alan T. Remaley, Marcella E. Comly
Publikováno v:
Journal of Biological Chemistry. 279:15571-15578
We have previously established that the ABCA1 transporter, which plays a critical role in the lipidation of extracellular apolipoprotein acceptors, traffics between late endocytic vesicles and the cell surface (Neufeld, E. B., Remaley, A. T., Demosky
Autor:
Ehud Goldin, Adele Cooney, Christine R. Kaneski, Surya N. Gupta, James R. Ellis, Stefanie Stahl, Roscoe O. Brady, Raphael Schiffmann
Publikováno v:
Human Mutation. 24:460-465
A patient with mucolipidosis-IV heterozygous for two mutations in MCOLN1 expressed only her father's cDNA mutation c.1207C>T predicting an R403C change in mucolipin. She inherited a 93bp segment from mitochondrial NADH dehydrogenase 5 (MTND5) from he
Autor:
Yunden Jinsmaa, Yehonatan Sharabi, David S. Goldstein, Patricia Sullivan, Daniel J. Gross, Adele Cooney
Publikováno v:
Neuroscience letters. 569
Parkinson disease (PD) features profound striatal dopamine depletion and Lewy bodies containing abundant precipitated alpha-synuclein. Mechanisms linking alpha-synucleinopathy with the death of dopamine neurons remain incompletely understood. One suc
Autor:
M E Comly, Joan Blanchette-Mackie, Silvia Santamarina-Fojo, Adele Cooney, Edward B. Neufeld, Nancy K. Dwyer, Mei Zhang, John A. Stonik, Alan T. Remaley, H. Bryan Brewer, Stephen J. Demosky
Publikováno v:
Journal of Biological Chemistry. 276:27584-27590
ABCA1, the ATP-binding cassette protein mutated in Tangier disease, mediates the efflux of excess cellular sterol to apoA-I and thereby the formation of high density lipoprotein. The intracellular localization and trafficking of ABCA1 was examined in