Disease relevance of Arsb

• Targeted disruption of the arylsulfatase B gene results in mice resembling the phenotype of mucopolysaccharidosis VI [1].
• Mucopolysaccharidosis VI (MPS VI) is a lysosomal storage disease with autosomal recessive inheritance caused by a deficiency of the enzyme arylsulfatase B (ASB), which is involved in degradation of dermatan sulfate and chondroitin 4-sulfate [1].
• Activity of some hepatic enzymes in schistosomiasis and concomitant alteration of arylsulfatase B [2].
• Arylsulfatase A, arylsulfatase B, and arylsulfatase Bv, in both normal liver and granulomas, were separated by anion-exchange column chromatography and differences in net charges of these enzymes were demonstrated by polyacrylamide gel electrophoresis [3].
• Phosphorylation on protein and carbohydrate moieties of a lysosomal arylsulfatase B variant in human lung cancer transplanted into athymic mice [4].

High impact information on Arsb

• Homozygous mutant animals exhibit ASB enzyme deficiency and elevated urinary secretion of dermatan sulfate [1].
• Cells derived from dorsal, intermediate, and ventral regions of the spinal cord all behaved similarly and gave rise to motor neuron-like cells when plated onto Ast-1 [5].
• Together, these results indicate that precursors in the murine spinal cord can be induced to differentiate into the motor neuron phenotype by factors produced by Ast-1 cells, suggesting that a similar factor(s) produced by cells akin to Ast-1 may regulate motor neuron differentiation in vivo [5].
• By using cells that expressed the lacZ reporter gene, it was shown that > 93% of cells present on the Ast-1 monolayers were motor neuron-like [5].
• Time-lapse analysis revealed that the precursors on the Ast-1 monolayers gave rise to neurons either directly or following a single cell division [5].

Chemical compound and disease context of Arsb

• After treatments with a nitrosourea (PCNU), mice bearing transplants of a large-cell carcinoma (ASB XIV) had complete remissions (CR) in 13 of 20 animals on a 15 mg/kg regimen, in 6 of 20 on an 8 mg/kg regimen, and in none of 10 on a 4 mg/kg regimen [6].
• A squamous cell carcinoma (ASB XIII) and a large cell carcinoma (ASB XIV) induced from mouse lung cells by chrysotile asbestos were established in serial transplant in BALB/c mice [7].

Biological context of Arsb

• Linkage of loci affecting a murine liver protein and arylsulfatase B to chromosome 13 [8].
• The latter enzyme was thermostable, resistant to inhibition by silver, completely inhibited by phosphate, displayed linear kinetics, and had a higher pH optimum than arylsulfatase A. Anionic arylsulfatase B also hydrolyzed chondroitin-4-SO4 heptasaccharide [9].
• We have performed a genetic linkage analysis to map pulmonary adenoma susceptibility (Pas) loci in an urethane-treated (A/J x Mus spretus) x C57BL/6J (ASB) interspecific testcross [10].
• Assignment of a gene for arylsulfatase B to human chromosome 5 using human-mouse somatic cell hybrids [11].

Anatomical context of Arsb

• As ocular and cardiac impairment are also clinically important manifestations of the MPS VI syndrome, the present study was initiated for detailed biochemical, histologic and functional analysis of cornea, optic nerve and heart in ASB-deficient mice [12].
• Biochemical evidence for GAG-storage could be obtained for liver, kidney, spleen and myocardium as well as for heart valves, cornea and optic nerve from ASB-deficient mice [12].
• Neuronal production was optimal under the conditions in which precursors were initially stimulated with basic fibroblast growth factor and then exposed to medium conditioned by an astrocytic cell line, Ast-1, in serum-free medium [13].
• In addition, the Ast-1 conditioned medium was shown to promote the survival of the neuroepithelial cells [14].
• Ast-1 cell monolayers were also found to provide a suitable substrate for the prolonged survival (at least 3 days in vitro) of neurofilament positive neurons obtained from E17 mice, whereas neurons plated onto NIH 3T3 cells were all dead after 2 days [14].

Associations of Arsb with chemical compounds

• This infection caused a significant (p < 0.001) increase in the activity of hepatic arylsulfatase B (ASB), aspartate transcarbamylases and gamma-glutamyl transpeptidase [2].
• Rodent and bovine arylsulfatase B hydrolyze 4-methylumbelliferyl sulfate (4MUS) 10- to 30-fold more efficiently than arylsulfatase A. Therefore, 4MUS grossly underestimates arylsulfatase A activity in the presence of excess arylsulfatase B [15].
• After daily oral administration of 10 mg/kg of all-trans retinoic acid in feed, there was 61%-81% inhibition (P less than 0.005) of ASB XIII [7].
• Growth inhibition of ASB XIV was 39% (P less than 0.02) after injections of all-trans retinoic acid, and 33% (P greater than 0.05) after trimethylmethoxyphenyl analog, ethyl ester [7].
• In vivo incorporation of tritiated leucine into C57BL/6J hepatic arylsulfatase B reached a maximum approximately 15 h after injection [16].

Other interactions of Arsb

• As-1 and Lth-1 were found to be closely linked using recombinant inbred (RI) strains [8].
• This isozyme is antigenically similar to cationic arylsulfatase B, displays a similar developmental profile, and can be converted to a form resembling the cationic species by prior treatment with neuraminidase [17].

Analytical, diagnostic and therapeutic context of Arsb

• Kidney, brain, and spleen arylsulfatase B electrofocusing patterns did not appear to differ between SM/J and C57BL/6J mice [18].
• Sephacryl S-300 gel filtration resolved anionic arylsulfatase B into 55 and 115 kd fractions [9].

References