Chitotriosidase Monoclonal Antibody
Product details
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],
Specifications
| Size | 50μL/100μL |
| Target | Chitotriosidase |
| Reactivity | Human |
| Applications | WB, ELISA |
| MW(Calculated) | 52kD |
| MW(Observed) | |
| Host Species | Mouse |
| Isotype | |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-1:2000; ELISA 1:10000; Not yet tested in other applications. |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Purification | Affinity purification |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | |
| Clonality | Monoclonal |
| Clone Number | 7E2 |
| Immunogen | Purified recombinant fragment of Chitotriosidase (aa22-137) expressed in E. Coli. |
| Specificity | Chitotriosidase Monoclonal Antibody detects endogenous levels of Chitotriosidase protein. |
| Gene Name | CHIT1 |
| Protein Name | Chitotriosidase-1 |
| Other Name | CHIT1; / Chitotriosidase-1; / Chitinase-1 |
| Species | Human |
| Gene ID | 1118 |
| Uniprot | Q13231, |
| Species.1 | Mouse |
| Gene ID.1 | |
| Uniprot.1 | Q9D7Q1 |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | Secreted. Lysosome. A small proportion is lysosomal. |
| Tissue Expression | Detected in spleen. Secreted by cultured macrophages. |
| Signaling_pathway | |
| Research Areas | >>Amino sugar and nucleotide sugar metabolism; / >>Metabolic pathways |
| 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 to the glycosyl hydrolase 18 family. Chitinase class II subfamily.,similarity:Contains 1 chitin-binding type-2 domain.,subcellular location:A small proportion is lysosomal.,subunit:Monomer.,tissue specificity:Detected in spleen. Secreted by cultured macrophages., |
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