Human GLB1 Matched Antibody Pair set for ELISA use
Capture Antibody: Mouse monoclonal anti-GLB1
Detection Antibody: Rabbit purified polyclonal anti-GLB1
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Antigen Detail | |
|---|---|
| Target | GLB1 |
| Description | GM1-gangliosidosis is a lysosomal storage disease that can be caused by a deficiency of β-galactosidase (GLB1). Some cases of Morquio syndrome B have been shown to be due to GLP1 mutations that cause patients to have abnormal elastic fibers. |
| Species Reactivity | Human |
Capture Antibody | |
|---|---|
| Capture Clonality | Monoclonal |
| Host Species | Mouse |
| Clone | IV01772 |
| Purification | Affinity chromatography |
| Buffer | PBS, pH 7.4, containing 0.1% Proclin 300 |
| Application | ELISA |
Detection Antibody | |
|---|---|
| Det-Clonality | Polyclonal |
| Host Species | Rabbit |
| Conjugation | Unconjugated |
| Purification | Affinity chromatography |
| Buffer | PBS, pH 7.4 |
General Product Property | |
|---|---|
| Product Overview | Human GLB1 Matched Antibody Pair set for ELISA use Capture Antibody: Mouse monoclonal anti-GLB1 Detection Antibody: Rabbit purified polyclonal anti-GLB1 |
| Specificity | Validated for detecting natural and recombinant human GLB1 |
| Research Area | Metabolism |
| Application | ELISA |
| Application Notes | Optimal dilutions should be determined experimentally by the user. |
| Form | Lyophilized |
| Storage | Avoid repeated freeze/thaw cycles. Store at 2-8°C for short term. Aliquot and store at -20°C for long term storage. |
| Size | 1 Set |
| Sample Type | Serum; Supernatant; Urine; Cell lysate; Plasma; Tissue Homogenates |
| Usage Statement | Available for Research Use Only. Not for use in therapeutic or diagnostic applications. |
Target Overview | |
|---|---|
| Gene Symbol | GLB1 |
| Full Name | Galactosidase Beta 1 |
| Background | This gene encodes a member of the glycosyl hydrolase 35 family of proteins. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed to generate the mature lysosomal enzyme. This enzyme catalyzes the hydrolysis of a terminal beta-linked galactose residue from ganglioside substrates and other glycoconjugates. Mutations in this gene may result in GM1-gangliosidosis and Morquio B syndrome. [provided by RefSeq, Nov 2015] |
| Alternative Names | Acid Beta-Galactosidase |
| Gene ID | 2720 |
| Uniprot ID | P16278 |
| GeneCards | GLB1 |
| HGNC | 4298 |
| Ensembl | ENSG00000170266 |
| OMIM | 611458 |
| Involvement in Disease | Diseases associated with GLB1 include Gm1-Gangliosidosis, Type I and Gm1-Gangliosidosis, Type Ii. |
| Function | Has no beta-galactosidase catalytic activity, but plays functional roles in the formation of extracellular elastic fibers (elastogenesis) and in the development of connective tissue. Seems to be identical to the elastin-binding protein (EBP), a major component of the non-integrin cell surface receptor expressed on fibroblasts, smooth muscle cells, chondroblasts, leukocytes, and certain cancer cell types. In elastin producing cells, associates with tropoelastin intracellularly and functions as a recycling molecular chaperone which facilitates the secretions of tropoelastin and its assembly into elastic fibers. |
| Related Pathways | Its related pathways are Innate Immune System and Keratan sulfate/keratin metabolism. |
For Research Use Only.