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GoPubMed Proteins lists recent and important papers and reviews for proteins. Page last changed on 19 Dec 2016.

Arylsulfatase B

arylsulfatase B
Top mentioned proteins: Transcription Factor DP1, sulfatase, HAD, ACID, CAN
Papers on arylsulfatase B
Lacritin and other autophagy associated proteins in ocular surface health.
Laurie et al., Hyderābād, India. In Exp Eye Res, Mar 2016
Mutation of arylsulfatase A, arylsulfatase B, ceroid-lipofuscinosis neuronal 3, mucolipin, or Niemann-Pick disease type C1 respectively underlie several diseases of apparently insufficient autophagic flux that affect the eye, including: metachromatic leukodystrophy, mucopolysaccharidosis type VI, juvenile-onset Batten disease, mucolipidosis IV, and Niemann-Pick type C associated with myelin sheath destruction of corneal sensory and ciliary nerves and of the optic nerve; corneal clouding, ocular hypertension, glaucoma and optic nerve atrophy; accumulation of 'ceroid-lipofuscin' in surface conjunctival cells, and in ganglion and neuronal cells; decreased visual acuity and retinal dystrophy; and neurodegeneration.
Fluorimetric assays for N-acetylgalactosamine-6-sulfatase and arylsulfatase B based on the natural substrates for confirmation of mucopolysaccharidoses types IVA and VI.
Gelb et al., Seattle, United States. In Clin Chim Acta, Jan 2016
METHODS: We developed new fluorimetric assays for N-acetylgalactosamine-6-sulfatase (GALNS) and arylsulfatase B (ARSB) based on the natural substrates, N-acetylgalactosamine-6-sulfate (and 4-sulfate), which have improved activity and specificity toward the relevant enzymes.
Effect of CFTR Modifiers on Arylsulfatase B Activity in Cystic Fibrosis and Normal Human Bronchial Epithelial Cells.
Tobacman et al., Chicago, United States. In Pulm Pharmacol Ther, Dec 2015
BACKGROUND: The enzyme Arylsulfatase B (ARSB; N-acetylgalactosamine 4-sulfatase), is required for degradation of sulfated glycosaminoglycans (GAGs) which accumulate in cystic fibrosis.
Long-term cognitive follow-up in children treated for Maroteaux-Lamy syndrome.
van der Ploeg et al., Rotterdam, Netherlands. In J Inherit Metab Dis, Nov 2015
Two patients, both with high baseline glycosaminoglycan levels in their urine and severe mutations in the arylsulfatase B gene, scored clearly lower than expected.
Decline in arylsulfatase B and Increase in chondroitin 4-sulfotransferase combine to increase chondroitin 4-sulfate in traumatic brain injury.
Tobacman et al., Chicago, United States. In J Neurochem, Aug 2015
In an established rat model of penetrating ballistic-like brain injury (PBBI), arylsulfatase B (ARSB; N-acetylgalactosamine 4-sulfatase) activity was significantly reduced at the ipsilateral site of injury, but unaffected at the contralateral site or in sham controls.
Improving arylsulfatase activity determination in dried blood spots: Screening and diagnostic approaches for Maroteaux-Lamy syndrome (MPS VI).
Silvestroff et al., Buenos Aires, Argentina. In Clin Chim Acta, Jul 2015
BACKGROUND: Mucopolysaccharidosis type VI can be screened by measuring the lysosomal arylsulfatase B (ARSB) residual enzyme activity in dried blood spots (DBS) using synthetic substrates.
Prevalence of anti-adeno-associated virus serotype 8 neutralizing antibodies and arylsulfatase B cross-reactive immunologic material in mucopolysaccharidosis VI patient candidates for a gene therapy trial.
Auricchio et al., Napoli, Italy. In Hum Gene Ther, Mar 2015
In the prospect of an AAV8-mediated, liver-directed gene therapy clinical trial for mucopolysaccharidosis VI (MPS VI), a lysosomal storage disorder caused by arylsulfatase B (ARSB) deficiency, we investigated in a multiethnic cohort of MPS VI patients the prevalence of neutralizing antibodies (Nab) to AAV8 and the presence of ARSB cross-reactive immunologic material (CRIM), which will either affect the efficacy of gene transfer or the duration of phenotypic correction.
Genetics of Plasminogen Activator Inhibitor-1 (PAI-1) in a Ghanaian Population.
Williams et al., Utrecht, Netherlands. In Plos One, 2014
Three variants associated with median PAI-1, the most significant of which was in the gene arylsulfatase B (ARSB) (p = 1.09 x 10(-7)).
Attenuated osteoarticular phenotype of type VI mucopolysaccharidosis: a report of four patients and a review of the literature.
Tylki-Szymańska et al., Warsaw, Poland. In Clin Rheumatol, 2014
Mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome, MPS VI, OMIM 253200) is caused by mutations in the gene coding for N-acetylgalactosamine-4-sulfatase (4-sulfatase, arylsulfatase B, ARSB, EC, a lysosomal enzyme involved in the degradation of dermatan sulfate (DS).
Enzyme replacement therapy with galsulfase for mucopolysaccharidosis VI: clinical facts and figures.
Harmatz, Oakland, United States. In Turk J Pediatr, 2010
Mucopolysaccharidosis VI (MPS VI) is an inheritable, clinically heterogeneous lysosomal storage disorder that develops due to a deficiency in the arylsulfatase B (ASB) enzyme.
Mucopolysaccharidosis VI.
Turbeville et al., Paris, France. In Orphanet J Rare Dis, 2009
Over 130 ARSB mutations have been reported, causing absent or reduced arylsulfatase B (N-acetylgalactosamine 4-sulfatase) activity and interrupted dermatan sulfate and chondroitin sulfate degradation.
Galsulfase: arylsulfatase B, BM 102, recombinant human arylsulfatase B, recombinant human N-acetylgalactosamine-4-sulfatase, rhASB.
In Drugs R D, 2004
Galsulfase [Aryplase, arylsulfatase B, BM 102, Naglazyme, rhASB, recombinant human N-acetylgalactosamine-4-sulfatase, recombinant human arylsulfatase B] is under development with BioMarin Pharmaceutical as an enzyme replacement therapy for the treatment of mucopolysaccharidosis (MPS) VI.
Bone-marrow transplantation in the Maroteaux-Lamy syndrome (mucopolysaccharidosis type VI). Biochemical and clinical status 24 months after transplantation.
McGovern et al., In N Engl J Med, 1985
A 13-year-old girl with the severe form of the Maroteaux-Lamy syndrome (mucopolysaccharidosis Type VI, arylsulfatase B deficiency) has had successful reconstitution with bone marrow from her HLA-MLC-matched sister who had normal arylsulfatase B activity.
Mucopolysaccharidosis in a cat with arylsulfatase B deficiency: a model of Maroteaux-Lamy syndrome.
Greenstein et al., In Science, 1977
These cells showed a deficiency in arylsulfatase B activity.
Metachromatic leukodystrophy: diagnosis with samples of venous blood.
Brady et al., In Science, 1968
Acid phosphatase and arylsulfatase B activities were normal.
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