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Chemical Compound Review:

P12 Sulfatide (2R,3R,4S,5R,6R)-3,4,5- trihydroxy-2...

Synonyms: AC1O5R9Z, 111682-15-6

Bach, G. et al., Marchesini, S. et al.

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Disease relevance of P12 Sulfatide

Cultured skin fibroblasts from normal individuals and healthy persons suffering from a pseudoarylsulfatase A deficiency (PD) degraded the P12-sulfatide, while in cells derived from a metachromatic leukodystrophy (MLD) patient it remained essentially intact [1].

High impact information on P12 Sulfatide

A fluorescent derivative of cerebroside sulfate (12-(1-pyrene)dodecanoyl-sphingosylgalactosyl-0-3-sulfate (P12-sulfatide) has been synthesized as a potential substrate for the determination of cerebroside sulfatidase (or arylsulfatase A) activity [1].

Gene context of P12 Sulfatide

When extracts of skin fibroblasts derived from normal individuals and patients with Maroteaux-Lamy (lacking arylsulfatase B) or metachromatic leukodystrophy (lacking arylsulfatase A) were used as source of enzyme, P12-sulfatide was hydrolyzed by the former two but not by the latter cell extract [2].

References

Diagnosis of arylsulfatase A deficiency in intact cultured cells using a fluorescent derivative of cerebroside sulfate. Bach, G., Dagan, A., Herz, B., Gatt, S. Clin. Genet. (1987) [Pubmed]
Synthesis of pyrene derivatives of cerebroside sulfate and their use for determining arylsulfatase A activity. Marchesini, S., Viani, P., Cestaro, B., Gatt, S. Biochim. Biophys. Acta (1989) [Pubmed]

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