Zobrazeno 1 - 7
of 7
pro vyhledávání: '"Lydia B. Daniels"'
Publikováno v:
Clinica Chimica Acta. 115:369-375
Three fluorometric leukocyte beta -glucosidase assays were compared for their ability to diagnose Gaucher's disease and identify carriers of the disorder: the acid beta-glucosidase assay of Beutler and Kuhl [2], a pH 5.5-sodium taurocholate-dependent
Publikováno v:
Archives of Neurology. 39:550-556
Using glucocerebroside labeled with carbon 14 as the substrate, we determined that homogenates of brain tissue from both neuropathic and nonneuropathic cases of Gaucher's disease were profoundly deficient (more than 85%) in glucocerebrosidase activit
Purification and characterization of a cytosolic broad specificity beta-glucosidase from human liver
Publikováno v:
Journal of Biological Chemistry. 256:13004-13013
A cytoplasmic beta-glucosidase has been isolated and purified 9,000-fold to homogeneity from the liver of a case of type 1 Gaucher's disease to a specific activity of 400,000 nmol/h/mg of protein. Although markedly elevated above control levels in th
Publikováno v:
Journal of Biological Chemistry. 259:1714-1719
Glucocerebrosidase from normal human spleen, and spleen from cases of neurologic (types 2 and 3) and nonneurologic (type 1) Gaucher's disease, was delipidated and inactivated by extraction from membranes with sodium cholate and ice-cold 1-butanol. Co
Publikováno v:
Journal of neuropathology and experimental neurology. 41(6)
This study explores the biochemical basis that may distinguish neurologic and nonneurologic forms of Gaucher's disease. Crude membrane preparations from spleens of controls and patients representing the three clinical categories of Gaucher's disease
Publikováno v:
Clinica chimica acta; international journal of clinical chemistry. 106(2)
To date, enzymatic diagnosis of Gaucher's disease via a fluorometric assay procedure which utilizes 4-methylumbelliferyl-β- d -glucopyranoside as a substrate has not been possible when liver serves as the source of enzyme since currently employed fl
Autor:
Michael Palmieri, Robert H. Glew, Charles S August, Giulio D'Angio, Peter D. Nowell, Lydia B. Daniels, William L Elkins
Publikováno v:
Pediatric Research. 18:236A-236A
A 19 year-old female with non-neuronopathic Gaucher's Disease had 3 BMT's from 2 partially HLA-matched, carrier, sibling donors. Pre-BMT glucocerebrosidase (glc-ase) activity in leukocytes and liver were 13 and 9% of controls respectively. BMT #1 was