Query author contributions in Reactome
Reactome depends on collaboration between our curation team and outside experts to assemble and peer-review its pathway modules. The integration of ORCID within Reactome enables us to meet a key challenge with authoring, curating and reviewing biological information by incentivizing and crediting the external experts that contribute their expertise and time to the Reactome curation process. More information is available at ORCID and Reactome.
If you have an ORCID ID that is not listed on this page, please forward this information to us and we will update your Reactome pathway records.
Details on Person Arylsulfatase A (ARSA) hydrolyses a sulfatide (cerebroside 3...
| Class:Id | Summation:1606838 |
|---|---|
| _displayName | Arylsulfatase A (ARSA) hydrolyses a sulfatide (cerebroside 3... |
| _timestamp | 2023-08-07 09:06:58 |
| created | [InstanceEdit:1606810] Jassal, B, 2011-09-27 |
| modified | [InstanceEdit:1661981] Jassal, B, 2011-10-14 [InstanceEdit:1861756] Jassal, B, 2011-10-31 [InstanceEdit:9840997] Stephan, Ralf, 2023-08-07 |
| text | Arylsulfatase A (ARSA) hydrolyses a sulfatide (cerebroside 3-sulfate, SM4) to form a cerebroside and sulfate Stinshoff & Jatzkewitz, 1975). Saposin B (PSAP(195-273)) is a promiscuous lipid-binding and transfer protein and an essential cofactor for the lysosomal hydrolysis of sulfatide by ARSA. It binds sulfatides in soluble stoichiometric complexes, which ARSA recognizes as substrates (Fischer & Jatzkewitz, 1975). ARSA is present in the lysosomal lumen and comprises two chains, components B and C, linked by disulfide bonds (Stein et al. 1989, Fujii et al. 1992). The conversion to 3-oxoalanine (formylglycine, FGly) of a cysteine residue is critical for catalytic activity in all eukaryotes (Chruszcz et al. 2003, Lukatela et al. 1998). Defects in ARSA are a cause of metachromatic leukodystrophy (MLD) (MIM:250100), characterized by lysosomal storage of cerebroside-3-sulfate in neural and non-neural tissues (Gieselmann et al. 1991, Polten et al. 1991). Arylsulfatase A activity is reduced in multiple sulfatase deficiency (MSD) (MIM:272200), a disorder characterized by decreased activity of sulfatases. The defect is due to the lack of post-translational modification of the critical cysteine needed for activity (Schmidt et al. 1995). In metachromatic leukodystrophy due to saposin B deficiency (MLDSAPB, MIM:249290) sulfatide accumulates because of the missing cofactor saposin B (Regis et al., 1999). |
| (summation) | [Reaction:1606807] ARSA removes sulfate from PSAP(195-273):Sulfatide [Homo sapiens] |
| [Change default viewing format] | |
No pathways have been reviewed or authored by Arylsulfatase A (ARSA) hydrolyses a sulfatide (cerebroside 3... (1606838)
