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. METHO...

The relation of lysosomal enzymes to mammary tumor regression was studied in the highly hormone-dependent MTW9 rat mammary carcinoma. Quantities of the enzymes were determined with the use of a histochemical modification of the single radial immunodiffusion method. Major findings were as follows, (a) Acid phosphatase, arylsulfatase, ßgalactosidase, and 0-glucuronidase significantly increased i...

FOX1 gene encoding a multicopper ferroxidase is vital in iron metabolism in the single-celled green algae Chlamydomonas reinhardtii. Molecular characteristics of this gene have been extensively studied. However, little information is available about its regulatory factors in C. reinhardtii. In this study, we isolated a regulatory mutant of FOX1 gene by using an arylsulfatase gene driven by the ...

Mucopolysaccharidosis VI (MPS VI), or Maroteaux-Lamy syndrome, is an autosomal recessive lysosomal storage disorder caused by deficient activity of the enzyme arylsulfatase B (ASB). Progressive accumulation of glycosaminoglycans (GAGs) in organs and tissues leads to the development of multisystem clinical manifestations. The presentation of MPS VI is genotypically and phenotypically diverse, wi...

Arylsulfatases A, B, and C, beta-galactosidase, and acid phosphatase were assayed in neuronal, astroglial, and oligodendroglial fractions isolated from adult rabbit and beef brains. The specific activities of all acid hydrolases were lower in beef cells compared to rabbit cells. The lysosomal enzymes of the rabbit neuronal fraction showed 10--25 time higher activities than the oligodendroglial ...

In 15 patients with Japanese cedar pollinosis and 10 healthy control subjects, levels of major basic protein (MBP), eosinophil cationic protein (ECP), and arylsulfatase B (As) in the nasal secretions were examined before and after challenge with Japanese cedar pollen extract. The MBP and ECP levels in the patients were significantly higher 30 min after challenge than those before challenge (P <...

The innate ability of Cephalosporium acremonium to use methionine preferentially over sulfate for synthesis of cephalosporin C can be influenced through mutation. Mutants of C. acremonium with altered capacity to utilize sulfate for synthesis of antibiotic were isolated and partially characterized with respect to the uptake of sulfate and the regulation of arylsulfatase.

Gangliosides bound to subcellular particles from rat brain were labelled by incubation of the particles (i) with CMP-N[(3)H]-acetylneuraminic acid and (ii) simultaneously, with CMP-N[(3)H]-acetylneuraminic acid and UDP-N-acetyl-[(14)C(1)]galactosamine or with CMP-N[(3)H]-acetylneuraminic acid and UDP-[U-(14)C]-galactose. Analysis of the labelled gangliosides showed that in (i), (a) the labellin...

Aromatic sulfonates (R-SO(3)(-)) can be used as sulfur sources by sulfate-starved bacteria in laboratory cultures and the corresponding phenols are excreted from the cells. The present study was conducted to demonstrate whether such desulfonation reactions also occur in sulfate-leached agricultural soil, where desulfonation of organic sulfur compounds may have agronomic importance as a S source...