Chitotriosidase is secreted by activated human macrophages and is markedly elevated in plasma of Gaucher disease patients. The expression of chitotriosidase occurs only at a late stage of differentiation of monocytes to activated macrophages in culture. Human macrophages can synthesize a functional chitotriosidase, a highly conserved enzyme with a strongly regulated expression. This enzyme may play a role in the degradation of chitin-containing pathogens. Several alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Jan 2012]
Function:
Degrades chitin, chitotriose and chitobiose. May participate in the defense against nematodes and other pathogens. Isoform 3 has no enzymatic activity.
Subcellular Location:
Secreted. Lysosome. A small proportion is lysosomal.
Tissue Specificity:
Detected in spleen. Secreted by cultured macrophages.
Similarity:
Belongs to the glycosyl hydrolase 18 family. Chitinase class II subfamily.
Contains 1 chitin-binding type-2 domain.
SWISS:
Q13231
Gene ID:
1118
Database links:
Entrez Gene: 1118 Human
Omim: 600031 Human
SwissProt: Q13231 Human
Unigene: 201688 Human
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