免疫原
Purified recombinant fragment of Chitotriosidase (aa22-137) expressed in E. Coli.
特异性
Chitotriosidase Monoclonal Antibody detects endogenous levels of Chitotriosidase protein.
组成（Formulation）
Ascitic fluid containing 0.03% sodium azide,0.5% BSA, 50%glycerol.
稀释比例
Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications.
纯化工艺（Immunogen）
Affinity purification
背景（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],
功能
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
其他名称
CHIT1; Chitotriosidase-1; Chitinase-1
Fields
>>Amino sugar and nucleotide sugar metabolism;>>Metabolic pathways
细胞定位
Secreted. Lysosome. A small proportion is lysosomal.
组织表达
Detected in spleen. Secreted by cultured macrophages.
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