• Target：
• Chitotriosidase

• Fields：
• >>Amino sugar and nucleotide sugar metabolism;>>Metabolic pathways

• Gene Name：
• CHIT1

• Protein Name：
• Chitotriosidase-1

• Human Gene Id：
• 1118

• Human Swiss Prot No：
• Q13231

• Mouse Swiss Prot No：
• Q9D7Q1

• Immunogen：
• Purified recombinant fragment of Chitotriosidase (aa22-137) expressed in E. Coli.

• Specificity：
• Chitotriosidase Monoclonal Antibody detects endogenous levels of Chitotriosidase protein.

• Formulation：
• Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

• Source：
• Monoclonal, Mouse

• Dilution：
• WB 1:500 - 1:2000. ELISA: 1:10000. Not yet tested in other applications.

• Purification：
• Affinity purification

• Storage Stability：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• Other Name：
• CHIT1;Chitotriosidase-1;Chitinase-1

• Molecular Weight(Da)：
• 52kD

• References：
• 1. Sarcoidosis Vasc Diffuse Lung Dis. 2007 Mar;24(1):59-64.
  2. Clin Biochem. 2007 Mar;40(5-6):365-9.

• Background：
• 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：
• catalytic activity:Random hydrolysis of N-acetyl-beta-D-glucosaminide (1->4)-beta-linkages in chitin and chitodextrins.,disease:Very high plasma levels of CHIT1 are found in patients with Gaucher disease type 1 (GD I). This can be used as diagnostic aid and to evaluate the success of treatment. Successful therapy brings the CHIT1 activity levels back to normal.,function:Degrades chitin and chitotriose. May participate in the defense against nematodes and other pathogens. Isoform 3 has no enzymatic activity.,polymorphism:A 24 bp duplication in exon 10 leads to the activation of an alternative splice site and the production of an inactive protein. About 6% of the population are deficient for CHIT1 activity, while 35% are carriers and show reduced enzyme levels. People with CHIT1 deficiency appear perfectly healthy.,similarity:Belongs to the glycosyl hydrolase 18 family.,similarity:Belongs

• Subcellular Location：
• Secreted. Lysosome. A small proportion is lysosomal.

• Expression：
• Detected in spleen. Secreted by cultured macrophages.

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