Molecular Classification
Enzyme, Glycosyl hydrolase family 18
Disease Roles
Lysosomal storage disordersCardiovascular diseasesNeurodegenerative conditions

Chitotriosidase 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 immunity
Chitin degradation
Immunomodulation

Disease Associations

Lysosomal storage disorders
Cardiovascular diseases
Neurodegenerative conditions
Chronic inflammatory states
Infections

Safety Considerations

No safety concerns listed

Associated Biomarkers

Biomarker
Elevated chitotriosidase activity in serum/plasma
Therapy adherence in cystinosis patients treated with cysteamine