3.1.6.8: cerebroside-sulfatase
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For detailed information about cerebroside-sulfatase, go to the full flat file.
Word Map on EC 3.1.6.8
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3.1.6.8
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metachromatic
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leukodystrophy
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sulfatide
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medicine
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lysosomal
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asa
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demyelinate
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sural
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pseudodeficiency
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nitrocatechol
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sulfatases
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molecular biology
- 3.1.6.8
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metachromatic
- leukodystrophy
- sulfatide
- medicine
- lysosomal
- asa
-
demyelinate
-
sural
-
pseudodeficiency
- nitrocatechol
-
sulfatases
- molecular biology
Reaction
Synonyms
ARS, ARSA, arylsulfatase A, arylsulfatase E, arylsulfatase-A, AS-A, ASA, cerebroside sulfatase, cerebroside sulfate sulfatase, Cerebroside-sulfatase, sulfatase, cerebroside
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General Information on EC 3.1.6.8 - cerebroside-sulfatase
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GENERAL INFORMATION 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
malfunction
metabolism
physiological function
additional information
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heteromerization of wild-type and misfolded endoplasmic reticulum-degraded arylsulfatase A polypeptides affects the quality control of wild-type arylsulfatase A subunits. Within a heteromer, the misfolded subunit exerts a dominant negative effect on the wild-type subunit. Mechanism, overview
malfunction
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metachromatic leukodystrophy, MLD, is a lysosomal storage disease caused by a deficiency of the lysosomal enzyme arylsulfatase A
malfunction
metachromatic leukodystrophy, MLD, is a rare inherited lysosomal storage disorder caused by the deficiency of arylsulfatase A, ARSA
malfunction
inherited deficiency of arylsulfatase A leads to metachromatic leukodystrophy, MLD, a disease involving demyelination in the central and peripheral nervous systems, with an estimated frequency of 1 in 40000 newborns. Inherited deficiency for arylsulfatase leads to lysosomal storage of sulfated compounds and to serious diseases such as growth retardation, heart failure, and demyelination in the central nervous system
metabolism
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arylsulfatase A catalyzes the first step in the intralysosomal degradation of the sphingolipid 3-O-sulfogalactosylceramide, briefly called sulfatide
metabolism
ArsA is involved in the modulation of a sort of signaling cascade, which affects the morphology of the cells, by binding to the HSPGs located on the cell surface
physiological function
ARSA is involved in the catabolism of membrane sulfatides into galactosylceramide
physiological function
ArsA plays a role in the components of the extracellular matrix. ArsA functions as a substrate on which cells adhere and form protrusions. Coating culture plates with recombinant mouse ArsA, rmArsA, stimulates adhesion of human microvascular endothelial cells to the plate followed by the formation of cell protrusions as well as lamellipodia. rmArsA affects the architecture of the cytoskeleton, with a high density of actin filaments localized to peripheral regions of the cells and the extension of bundles of microtubules into the tips of cellular protrusions. rmArsA also affects the distribution pattern of the cell adhesion-associated proteins, integrin alpha2beta1, and paxillin. rmArsA seems to modulate signaling of basic fibroblast growth factor stimulating cytoskeletal rearrangement
