نتایج جستجو برای: globoid cell leukodystrophy

تعداد نتایج: 1684780  

Journal: :Journal of bacteriology 2001
B Härtl W Wehrl T Wiegert G Homuth W Schumann

The Bacillus subtilis lacA gene, coding for beta-galactosidase, has been explored as a new site able to accept DNA sequences from nonreplicating delivery vectors. Two such delivery expression vectors have been constructed and shown to be useful in obtaining regulated expression from the chromosomal location. In another experiment, it was shown that the integration of a regulatory gene at the la...

2013
Rodrigo P. P. Almeida Kent M. Daane Vaughn A. Bell G. Kai Blaisdell Monica L. Cooper Etienne Herrbach Gerhard Pietersen

Grapevine leafroll disease (GLD) is caused by a complex of vector-borne virus species in the family Closteroviridae. GLD is present in all grape-growing regions of the world, primarily affecting wine grape varieties. The disease has emerged in the last two decades as one of the major factors affecting grape fruit quality, leading to research efforts aimed at reducing its economic impact. Most r...

2015
Chris H. Hill Randy J. Read Janet E. Deane

The saposins are essential cofactors for the normal lysosomal degradation of complex glycosphingolipids by acid hydrolase enzymes; defects in either saposin or hydrolase function lead to severe metabolic diseases. Saposin A (SapA) activates the enzyme β-galactocerebrosidase (GALC), which catalyzes the breakdown of β-D-galactocerebroside, the principal lipid component of myelin. SapA is known to...

2013
Xing-Li Meng Yoshikatsu Eto Raphael Schiffmann Jin-Song Shen

Krabbe disease is a devastating neurodegenerative lysosomal storage disorder caused by a deficiency of β-galactocerebrosidase (GALC). Gene therapy is a promising therapeutic approach for Krabbe disease. As the human brain is large and it is difficult to achieve global gene transduction, the efficacy of cross-correction is a critical determinant of the outcome of gene therapy for this disease. W...

Journal: :Molecular biology and evolution 1985
D R Cavener

The glucose dehydrogenase gene (Gld) in Drosophila melanogaster exhibits a unique spatial and temporal pattern of expression. GLD expression switches from a non-sex-limited state at the pupal stage to a male-limited state at the adult stage. At the adult stage, the enzyme is restricted to the ejaculatory duct. Within the genus Drosophila, the ejaculatory duct has undergone a simple morphologica...

Journal: :Organic & biomolecular chemistry 2007
Kim L Curtis Emma L Evinson Sandeep Handa Kuldip Singh

Three diastereoisomers of 3-amino-4-hydroxy-2-(hydroxymethyl)pyrrolidine have been synthesised by a divergent route starting from trans-4-hydroxy-L-proline. Regio- and stereoselective introduction of the 3-amino and 4-hydroxyl functional groups was achieved using either a tethered aminohydroxylation reaction or by employing intra- and intermolecular epoxide-opening strategies. Preliminary biolo...

Journal: :Applied microbiology 1973
R Rosner

The PathoTec "Rapid I-D System" and two experimental test strips for ornithine decarboxylase and beta-galactosidase have been evaluated for accuracy and ability to identify 1,252 members of the Enterobacteriaceae obtained from fresh clinical specimens. Accuracy of identification with the commercially available test system was 94.7%; this level increased to 98.5% with the addition of the two exp...

Journal: :Journal of bacteriology 1971
H Soreq R Kaplan

Inducible and constitutive beta-galactosidase formation and radioactive amino acid incorporation were measured in cells recovering from various treatments which inhibit protein synthesis in the cell. Undelayed beta-galactosidase formation was found in stringent auxotrophs recovering from amino acid starvation, in cells recovering from glycerol or potassium starvation, and in bacteria recovering...

Journal: :Genes & development 1988
A M Boulet M P Scott

Antennapedia (Antp), a homeotic gene of Drosophila required for proper differentiation of the thorax of the fly, is expressed in complex spatial patterns during development. The gene is greater than 100 kb long and has two independently regulated promoters. To characterize cis-acting regulatory elements responsible for the expression pattern, fusions of the Antp promoter 2 cap site and upstream...

2006
Shan Lin Tianquan Lü Wenwu Cao

A generalized Ginzburg–Landau–Devonshire (GLD) theory is used to study the properties of ferroelectric thin films sandwiched between two metal electrodes with a first-order phase transition. By taking into account the effect of the imperfect surface layer and a natural boundary condition, the temperature and film-thickness dependence of the spontaneous polarization and the dielectric susceptibi...

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