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
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
Affinity purification
其他名称
CHIT1; Chitotriosidase-1; Chitinase-1
背景
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
