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positive regulation of neuron projection development
response to pH
chondroitin sulfate catabolic process
lysosomal transport
metabolism
response to methylmercury
lysosome organization
neutrophil degranulation
Sources:Amigo / QuickGO
Orthologs
Species
Human
Mouse
Entrez
411
11881
Ensembl
ENSG00000113273
ENSMUSG00000042082
UniProt
P15848
P50429
RefSeq (mRNA)
NM_000046 NM_198709
NM_009712
RefSeq (protein)
NP_000037 NP_942002
NP_033842
Location (UCSC)
Chr 5: 78.78 – 78.99 Mb
Chr 13: 93.91 – 94.08 Mb
PubMed search
[3]
[4]
Wikidata
View/Edit Human
View/Edit Mouse
arylsulfatase B
Crystallographic structure of putative tetrameric arylsulfatase from Escherichia coli.[5]
Identifiers
Symbol
ARSB
NCBI gene
411
HGNC
714
OMIM
253200
RefSeq
NM_000046
UniProt
P15848
Other data
EC number
3.1.6.12
Locus
Chr. 5 p11-q13
Search for
Structures
Swiss-model
Domains
InterPro
Galsulfase INN: galsulfase
Clinical data
Trade names
Naglazyme
Other names
Aryplase
AHFS/Drugs.com
Professional Drug Facts
License data
US DailyMed: Galsulfase
Pregnancy category
AU: B3[6]
Routes of administration
Intravenous
ATC code
A16AB08 (WHO)
Legal status
Legal status
US: ℞-only
EU: Rx-only
In general: ℞ (Prescription only)
Identifiers
CAS Number
552858-79-4
DrugBank
DB01279
UNII
59UA429E5G
KEGG
D06565
ChEMBL
ChEMBL1201822
Chemical and physical data
Formula
C2529H3843N689O716S16
Molar mass
55868.63 g·mol−1
Arylsulfatase B (N-acetylgalactosamine-4-sulfatase, chondroitinsulfatase, chondroitinase, acetylgalactosamine 4-sulfatase, N-acetylgalactosamine 4-sulfate sulfohydrolase, EC 3.1.6.12) is an enzyme associated with mucopolysaccharidosis VI (Maroteaux–Lamy syndrome).
Arylsulfatase B is among a group of arylsulfatase enzymes present in the lysosomes of the liver, pancreas, and kidneys of animals. The purpose of the enzyme is to hydrolyze sulfates in the body. ARSB does this by breaking down glycosaminoglycans (GAGs), which are large sugar molecules in the body. ARSB targets two GAGs in particular: dermatan sulfate and chondroitin sulfate.[7]
Over 130 mutations to ARSB have been found, each leading to a deficiency in the body. In most cases, the mutation occurs on a single nucleotide in the sequence. An arylsulfatase B deficiency can lead to an accumulation of GAGs in lysosomes,[7] which in turn can lead to mucopolysaccharidosis VI.
Used as a pharmaceutical drug, the enzyme is known under the International Nonproprietary Name galsulfase and is sold under the brand name Naglazyme.[8][9][10] Galsulfase was approved for medical use in the United States in May 2005 and in European Union in January 2006.[11][10] Galsulfase is indicated for long-term enzyme-replacement therapy in people with a confirmed diagnosis of mucopolysaccharidosis VI (MPS VI; N-acetylgalactosamine-4-sulfatase deficiency; Maroteaux-Lamy syndrome).[10]
Kyte-Doolittle hydropathy plot: enlarge for better viewing
^ abcGRCh38: Ensembl release 89: ENSG00000113273 – Ensembl, May 2017
^ abcGRCm38: Ensembl release 89: ENSMUSG00000042082 – Ensembl, May 2017
^"Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^"Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^PDB: 3ED4; Patskovsky Y, Ozyurt S, Gilmore M, Chang S, Bain K, Wasserman S, Koss J, Sauder MJ, Burley SK, Almo SC (2010). "Crystal structure of putative arylsulfatase from Escherichia coli". Worldwide Protein Data Bank. doi:10.2210/pdb3ed4/pdb.
^ ab"Galsulfase (Naglazyme) Use During Pregnancy". Drugs.com. 11 December 2019. Retrieved 23 April 2020.
^ abU.S. National Library of Medicine. "ARSB", Genetics Home Resource, 7 November 2010, Retrieved 22 November 2010
^Kim KH, Decker C, Burton BK (March 2008). "Successful management of difficult infusion-associated reactions in a young patient with mucopolysaccharidosis type VI receiving recombinant human arylsulfatase B (galsulfase [Naglazyme])". Pediatrics. 121 (3): e714–7. doi:10.1542/peds.2007-0665. PMID 18250117. S2CID 3298398.
^World Health Organization (2005). "International nonproprietary names for pharmaceutical substances (INN) : recommended international nonproprietary names (Rec. INN) : list 54". WHO Drug Information. 19 (3): 255. hdl:10665/73503.
^ abc"Naglazyme EPAR". European Medicines Agency (EMA). 17 September 2018. Retrieved 23 April 2020. This article incorporates text from this source, which is in the public domain.
^"Drug Approval Package: Naglazyme (Galsulfase) NDA #125117". U.S. Food and Drug Administration (FDA). 9 September 2005. Retrieved 23 April 2020.
for sulfonated hexoses. Types include: Arylsulfatase A (also known as "cerebroside-sulfatase") ArylsulfataseB (also known as "N-acetylgalactosamine-4-sulfatase")...
Arylsulfatase A (or cerebroside-sulfatase) is an enzyme that breaks down sulfatides, namely cerebroside 3-sulfate into cerebroside and sulfate. In humans...
09.009. S2CID 2346727. Tobacman JK (June 2003). "Does deficiency of arylsulfataseB have a role in cystic fibrosis?". Chest. 123 (6): 2130–2139. doi:10...
as the first medication-based intervention to treat phenylketonuria ArylsulfataseB (branded as Naglazyme) (galsulfase), a recombinant protein therapeutic...
recombinant human N-acetylgalactosamine 4-sulfatase (recombinant human arylsulfataseB or rhASB) and follow-on, open-label extension study. The Journal of...
[citation needed] Arylsulfatase A is activated by saposin B (Sap B), a non-enzymatic proteinaceous cofactor. When the arylsulfatase A enzyme level is...
Arylsulfatase L is an enzyme that, in humans, is encoded by the ARSL gene. Arylsulfatase L is a member of the arylsulfatase subfamily of sulfatase enzymes...
weeks (slow growers). Arylsulfatase test Arylsulfatase enzyme is present in most mycobacteria. The rate by which arylsulfatase enzyme breaks down phenolphthalein...
sulfatase (STS), or steryl-sulfatase (EC 3.1.6.2), formerly known as arylsulfatase C, is a sulfatase enzyme involved in the metabolism of steroids. It...
Defects Foundation: 116–21. Restivo A, Sarkozy A, Digilio MC, Dallapiccola B, Marino B (February 2006). "22q11 deletion syndrome: a review of some developmental...
as saposin B must be present. Saposin B extracts sulfatide from the membrane, which makes it accessible to arylsulfatase A. Arylsulfatase A can then hydrolyze...
them "lysosomes" to reflect their digestive properties. The same year, Alex B. Novikoff from the University of Vermont visited de Duve's laboratory, and...
of mutations in three different ARSA alleles that encode the enzyme arylsulfatase A (ASA or sometimes ARSA), also called sulfatide sulfatase. ASA is responsible...
been shown to interact with Pregnancy-associated plasma protein A. Arylsulfatase GRCh38: Ensembl release 89: ENSG00000186652 – Ensembl, May 2017 GRCm38:...
Saposin B was the first to be discovered and was found to be required as a heat-stable factor for hydrolysis of sulfatides by arylsulfatase A. It is...
nonlinear pharmacokinetics, while micafungin (hepatically metabolized by arylsulfatase, catechol O-methyltransferase, and hydroxylation) and anidulafungin...
Miech, C., Dierks, T., Selmer, T., Figura, K. von and Schmidt, B. (1998) Arylsulfatase from Klebsiella pneumoniae Carries a Formylglycine Generated from...
cells transduced ex vivo using a lentiviral vector encoding the human arylsulfatase A gene), a gene therapy for the treatment of children with the "late...
antibiotic p-aminosalicylate. M. abscessus has also been shown to produce arylsulfatase, but not of nitrate reductase and Tween 80 hydrolase. It shows a negative...
of steroid and other hormone levels by microbial glucuronidases and arylsulfatase profoundly impacted the performance of the composite entity. Following...
at 35 °C. Members also demonstrate a positive result for the 3 day arylsulfatase test. The genome size for Mycobacteroides species are between 4.5 and...
Global Information
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