A 10-year-old speechless, mentally deficient male, with low arylsulfatase A (ARSA) activity, and presumably, methachromatic leukodystrophy, underwent genetic evaluation. As the clinical picture was not compatible with this diagnosisan ARSA gene and chromosome analysis were performed, showing the presence of a pseudodeficiency ARSA allele and a de novo apparently balanced t(16;22)(p11.2;q13) tra...

Tracheal smooth muscle obtained from ovalbumin-sensitized guinea pigs contracted with micromolar concentrations of tartrazine and indomethacin. The contractions were slightly reduced by clemastine, but were completely blocked by the SRS-A antagonist FPL 55712. Arylsulfatase B, which is an enzyme that inactivates SRS-A, also abolished the contractions induced by tartrazine. Contractions induced ...

The lysosomal storage disorders are a group of diseases that typified by an accumulation waste products in the lysosomes. Mucopolysaccharidoses due to diverse enzyme deficiencies. Ms HT was 2 years and 5 months old when she presented our metabolic bone clinic with clinical features were suggestive genetic syndrome associated disease. urine GAG spot test positive. MPS screen identified reduction...

BACKGROUND Diabetes mellitus (DM) is characterized by the overproduction of the reactive oxygen species which affects the integrity of the lysosomal membrane affecting lysosomal enzymes. The effect of these species is blocked by some natural products as antioxidants. In the current study, groups of normal and streptozotocin (STZ)-induced diabetic rats were treated by Nigella sativa (NS), olive ...

Background: Mucopolysaccharidosis type-VI (MPS-VI), which is inherited as an autosomal recessive trait, results from the deficiency of N-acetylgalactosamine 4-sulfatase (arylsulfatase B) activity and the lysosomal accumulation of dermatan sulfate. In this study, ARSB mutation analysis was performed on three unrelated patients who were originally from the West Azerbaijan province of Iran. Method...

Arylsulfatase activities (96-h reaction) of various strains of Mycobacterium avium and M. intracellulare, as identified by a DNA probe test, were measured. The enzyme activities of M. avium strains were significantly lower [corrected] than those of M. intracellulare strains (P less than 0.005 to P less than 0.025). The enzyme activities did not vary with serovar; that is, the activities of sero...

A lectin was isolated from root tubers of winter aconite (Eranthis hyemalis) by affinity chromatography on fetuin-agarose, and it was partially characterized with respect to its biochemical, physicochemical and carbohydrate-binding properties. The Eranthis hyemalis lectin is a dimeric protein (Mr 62000) composed of two different subunits of Mr 30000 and 32000, held together by disulphide bonds....

Appropriately using phosphorus (P) for the fertilization of sugarcane is critical to achieving high productivity because it one most limiting nutrients in agricultural cropping systems. Thus, objective this research was evaluate morphological, biochemical, and yield responses sugarcane, soil microbial–chemical properties, under use organomineral fertilizer (OF) associated or not with Bacillus v...