Dictionary > Cerebroside-sulfatase

Cerebroside-sulfatase

Definition
noun
An enzyme that catalyzes the hydrolysis of cerebroside 3-sulfate (sulfatide) to yield a cerebroside and inorganic sulfate
Supplement
Cerebroside-sulfatase is an enzyme that catalyzes the chemical reaction:
cerebroside 3-sulfate + H2O ⇌ cerebroside + sulfate. It belongs to sulfatases, a group of enzymes that catalyzes the hydrolysis of sulfuric esters to the corresponding alcohols plus inorganic sulfate. They are important for their role in the degradation of sulfated glycosaminoglycans and glycolipids in the lysosome. In particular, cerebroside-sulfatase hydrolyzes galactose-3-sulfate residues in certain lipids.
Arylsulfatase a is regarded as the heat-labile component of the cerebroside-sulfatase. A marked deficiency of this component has been observed in all forms of metachromatic leukodystrophy. Metachromatic leukodystrophy is a metabolic disorder caused by a defective cerebroside-sulfatase due to a mutation involving an autosomal recessive gene. Without a functional cerebroside-sulfatase, the result is a failure to degrade sulfatides in the lysosome. Sulfatides are cerebroside sulfuric esters containing one or more sulfate groups in the sugar portion of the molecule. The presence of sulfatides in excess may lead to metachromatic leukodystrophy. In particular, sulfatide compounds (galactosyl sulfatidates) accumulate in the lysosomes in the cells of nerve tissues. This leads to the demyelination of the central nervous system and the peripheral nervous system. The development of the myelin (a fatty covering around the nerve fibers) is affected.
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