نتایج جستجو برای: globoid cell leukodystrophy

تعداد نتایج: 1684780  

Journal: :Applied and environmental microbiology 1985
H Hirata S Negoro H Okada

By cloning the beta-galactosidase gene of Bacillus stearothermophilus IAM11001 (ATCC 8005) into Bacillus subtilis, enzyme production was enhanced 50 times. beta-Galactosidase could be purified to 80% homogeneity by incubating the cell extract of B. subtilis at 70 degrees C for 15 min, followed by centrifugation to remove the denatured proteins. Because of its heat stability and ease of producti...

2009
Paul J. van Staden

The generalized lambda distribution (GLD) is a flexible distribution for statistical modelling, but existing estimation methodologies for the GLD are computationally difficult, rendering the GLD impractical for many practitioners. We derive a parameterization of the GLD with closed-form expressions for the method of L-moment estimators. A numerical example involving the age of coronary heart di...

Journal: :Journal of bacteriology 1973
R Schleif W Hess S Finkelstein D Ellis

After addition of l-arabinose to growing Escherichia coli, the l-ribulokinase (EC 2.7.1.16) and l-arabinose isomerase (EC 5.3.1.4) first appear at about 0.7 and 1.4 min, respectively. These times are consistent with the distances of the genes from the ribonucleic acid polymerase initiation site in the operon. The kinetics of appearance of these enzymes as well as those of beta-galactosidase (EC...

Journal: :Journal of clinical microbiology 2001
C Dando K E Gabriel J S Remington S F Parmley

A simple and efficient method using transgenic Toxoplasma gondii tachyzoites expressing beta-galactosidase was developed for detection of specific antibodies against the parasite in sera of patients. The titers obtained with the new test were similar to those obtained with the Sabin-Feldman dye test run in parallel. Although significant changes in endpoint titers were not observed when sera dra...

Journal: :Endocrinologia japonica 1990
Y Nishi

Two young girls with hirsutism and premature pubarche showed nonclassical 3 beta-hydroxysteroid dehydrogenase (3 beta-HSD) deficiency. Post-ACTH increased serum delta 5-17-hydroxypregnenolone and increased ratio of delta 5-17-hydroxypregnenolone/17-hydroxyprogesterone are the most sensitive indicators of nonclassical 3 beta-HSD deficiency. Nonclassical 3 beta-HSD deficiency may not be uncommon,...

Journal: :The Journal of neuroscience : the official journal of the Society for Neuroscience 2002
Masako Taniike Ikuko Mohri Naomi Eguchi Carsten T Beuckmann Kinuko Suzuki Yoshihiro Urade

The genetic demyelinating mouse "twitcher" is a model of the human globoid cell leukodystrophy, caused by galactosylceramidase (GALC) deficiency. Demyelination in the twitcher brain is secondary to apoptotic death of oligodendrocytes (OLs). Lipocalin-type prostaglandin (PG) D synthase (L-PGDS), a protein expressed in mature OLs, was progressively upregulated in twitcher OLs; whereas expression ...

Journal: :Journal of lipid research 2003
Keiko Tadano-Aritomi Junko Matsuda Hirokazu Fujimoto Kunihiko Suzuki Ineo Ishizuka

Sphingolipid activator proteins (saposins A, B, C, and D) are derived from a common precursor protein (prosaposin) and specifically activate in vivo degradation of glycolipids with short carbohydrate chains. A mouse model of prosaposin deficiency (prosaposin-/-) closely mimics the human disease with an elevation of multiple glycolipids. The recently developed saposin A-/- mice showed a chronic ...

2015
M. Zerkaoui I. Ratbi B. Castellotti C. Gellera J. Lyahyai Y. Kriouile A. Sefiani

BACKGROUND Krabbe disease (KD) or globoid cell leukodystrophy is an autosomal recessive lysosomal disorder, which affects metabolic and neurologic systems. This pathology has different forms. Infantile onset is about 85% to 90% of individuals with Krabbe disease. Disorder's onset is characterized, in early childhood, by hyperirritability, psychomotor deterioration associated to episodes of feve...

Journal: :Antimicrobial agents and chemotherapy 2010
Anita Nielsen Kristian F Nielsen Dorte Frees Thomas O Larsen Hanne Ingmer

We present a simple assay to examine effects of compounds on virulence gene expression in the human pathogen Staphylococcus aureus. The assay employs transcriptional reporter strains carrying lacZ fused to central virulence genes. Compounds affecting virulence gene expression and activity of the agr locus are scored based on color change in the presence of a chromogenic beta-galactosidase subst...

Journal: :The Journal of chemical physics 2013
Andrew D Baczewski Stephen D Bond

Generalized Langevin dynamics (GLD) arise in the modeling of a number of systems, ranging from structured fluids that exhibit a viscoelastic mechanical response, to biological systems, and other media that exhibit anomalous diffusive phenomena. Molecular dynamics (MD) simulations that include GLD in conjunction with external and/or pairwise forces require the development of numerical integrator...

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