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Chitotriosidase

Molecular classification: Enzyme, Glycosyl hydrolase family 18

Overview

Chitotriosidase is an enzyme (EC 3.2.1.14) and the major active chitinase in humans, primarily produced by activated macrophages. It plays a significant role in innate immunity, particularly as a defense mechanism against chitin-containing organisms such as fungi and protozoa. Elevated chitotriosidase activity serves as a biomarker for macrophage activation and is used clinically to monitor diseases characterized by increased macrophage burden or activation.

Mechanism of action

Endochitinase, cleaving internal β-(1→4)-glycosidic bonds within chitin polymers; also exhibits transglycosylation activity.

Biological functions

Innate immunityChitin degradationImmunomodulation

Disease associations

Lysosomal storage disordersCardiovascular diseasesNeurodegenerative conditionsChronic inflammatory statesInfections

Biomarkers

Elevated chitotriosidase activity in serum/plasmaTherapy adherence in cystinosis patients treated with cysteamine

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