Skip to content

DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap

Data

Loading the map…

Human metabolism

Reactome's human metabolism drawn as one network. Each molecule has one home node; widely shared molecules also appear as dashed copies in other territories, linked from their details. .

Territories

Select a territory to bring it forward.

Reading the map

Metabolite; its structure appears as you zoom in
Reaction, arrow toward the product
Cofactors and co-substrates are written beside the reaction when you zoom in
Dashed ring: a copy of a widely shared molecule or cofactor in another territory or pathway
Compartment transfer of the molecule it is linked to
Binding, release or complex event; its proteins are named in its details
Outline: reactions mostly in the mitochondrion

Arrows follow Reactome's curated reaction direction.

ARSA removes sulfate from PSAP(195-273):Sulfatide

H2O + PSAP(195-273):Sulfatide:PE → GalCer + SO4(2-) + PSAP(195-273) dimer + PE 30,32,34 + H+

Reactome: R-HSA-1606807
Compartment: lysosomal lumen
Rhea: None recorded

Inputs

H2OCHEBI:15377, H2O, lysosomal lumen
PSAP(195-273):Sulfatide:PER-HSA-9840996, lysosomal lumen

Outputs

GalCerCHEBI:36498, C10H17NO8R2, lysosomal lumen
SO4(2-)CHEBI:16189, O4S, lysosomal lumen
PSAP(195-273) dimerR-HSA-9840482, lysosomal lumen
PE 30,32,34R-ALL-9840939, lysosomal lumen
H+CHEBI:15378, H, lysosomal lumen

Catalysed by

cerebroside-sulfatase activity of active ARSA:Ca2+ [lysosomal lumen]EC 3.1.6.8

Literature

Cloning and expression of human arylsulfatase A J Biol Chem, 1989
Proteolytic processing of human lysosomal arylsulfatase A Biochim Biophys Acta, 1992
Crystal structure of a covalent intermediate of endogenous human arylsulfatase A J Inorg Biochem, 2003
Crystal structure of human arylsulfatase A: the aldehyde function and the metal ion at the active site suggest a novel mechanism for sulfate ester hydrolysis Biochemistry, 1998
Mutations in the arylsulfatase A pseudodeficiency allele causing metachromatic leukodystrophy Am J Hum Genet, 1991
Molecular basis of different forms of metachromatic leukodystrophy N Engl J Med, 1991