Results 291 to 300 of about 1,020,842 (327)
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Beta-Galactosidase Deficiency in the Hurler Syndrome
New England Journal of Medicine, 1969Abstract A deficiency of β-galactosidase (pH 5.0) was found in frozen tissues (brain, liver, kidney and spleen) from 10 patients with Hurler's syndrome (Types 1–3). The diminished activity of this enzyme was demonstrated with the use of nitrophenyl-galactosides as well as ganglioside GM1 and a "keratan sulfate-like" mucopolysaccharide.
Mae Wan Ho +6 more
openaire +3 more sources
, 2018
Access to streamlined computational resources remains a significant bottleneck for new users of cryo-electron microscopy (cryo-EM). To address this, we have developed tools that will submit cryo-EM analysis routines and atomic model building jobs ...
Cianfrocco +3 more
semanticscholar +1 more source
Access to streamlined computational resources remains a significant bottleneck for new users of cryo-electron microscopy (cryo-EM). To address this, we have developed tools that will submit cryo-EM analysis routines and atomic model building jobs ...
Cianfrocco +3 more
semanticscholar +1 more source
Cold Spring Harbor Protocols, 2010
When a transient or stable transfection assay is developed for a promoter, a primary objective is to quantify promoter strength. Because transfection efficiency in such assays can be low, promoters are commonly fused to heterologous reporter genes that ...
S. Smale
semanticscholar +1 more source
When a transient or stable transfection assay is developed for a promoter, a primary objective is to quantify promoter strength. Because transfection efficiency in such assays can be low, promoters are commonly fused to heterologous reporter genes that ...
S. Smale
semanticscholar +1 more source
Immobilization of mold beta-galactosidase
Collection of Czechoslovak Chemical Communications, 1988We immobilized beta-galactosidase (EC 3.2.1.23) from the mold Aspergillus oryzae by various methods on the derivatives of bead cellulose manufactured by Czechoslovak industry. The activity, specific activity and operational stability of the immobilized enzyme were determined.
Alexandra Proskova +2 more
openaire +2 more sources
Beta-Galactosidase Staining in the Skeleton
2014The lacZ gene, encoding for the β-galactosidase enzyme, is widely used as a reporter gene in bone biology due to the ease of visualization in situ on whole-mount or on tissue sections. In this protocol we provide detailed methods for visualizing this reporter gene for both in vivo and in vitro studies.
Jian Q. Feng, X. L. Han
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Generalized Gangliosidosis: Beta-Galactosidase Deficiency
Science, 1968A profound deficiency (10- to 30-fold) of β-galactosidase activity was found in tissues (liver, spleen, kidney, and brain) from two patients with generalized gangliosidosis; this deficiency is demonstrated as a failure to cleave both p -nitrophenyl-β-D-galactopyranoside and ganglioside GM 1 ...
Shintaro Okada, John S. O'Brien
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Angewandte Chemie, 2010
This thesis deals with the design, formation and evaluation of dynamic systems constructed by means of sulfur-containing reversible reactions, in organic and aqueous media and under mild conditions.
Rémi Caraballo +4 more
semanticscholar +1 more source
This thesis deals with the design, formation and evaluation of dynamic systems constructed by means of sulfur-containing reversible reactions, in organic and aqueous media and under mild conditions.
Rémi Caraballo +4 more
semanticscholar +1 more source
Three cases of beta-galactosidase deficiency
Klinische Pädiatrie, 1981A clinical description of three cases of beta-galactosidase deficiency is presented. Two cases are classical for infant type GM1-gangliosidosis, the third is characterised by dysostosis multiplex with growth retardation and of normal intelligence. Laboratory data revealed mucopolysaccharides in the urine of all described patients in a high level of ...
Ewa Pronicka +3 more
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Biochemistry, 1995
Human lysosomal beta-galactosidase is organized as a 680-kDa complex with cathepsin A (also named carboxypeptidase L and protective protein), which is necessary to protect beta-galactosidase from intralysosomal proteolysis. To understand the molecular mechanism of beta-galactosidase protection by cathepsin A, we defined the structural organization of ...
Marc-André Elsliger +3 more
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Human lysosomal beta-galactosidase is organized as a 680-kDa complex with cathepsin A (also named carboxypeptidase L and protective protein), which is necessary to protect beta-galactosidase from intralysosomal proteolysis. To understand the molecular mechanism of beta-galactosidase protection by cathepsin A, we defined the structural organization of ...
Marc-André Elsliger +3 more
openaire +3 more sources
A dimer-dimer binding region in .beta.-galactosidase
Biochemistry, 1979alpha Complementation in beta-galactosidase is the restoration of enzyme activity by addition of the alpha donor CNBr2, from amino acid residues 3--92 of the polypeptide, to inactive M15 protein from the lacZ deletion mutant strain M15. M15 protein lacks residues 11--41 and is a dimer; the active complex, like native beta-galactosidase, is tetrameric ...
Irving Zabin, Franco Celada
openaire +3 more sources