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Description
beta Galactosidase/GLB1 Recombinant Rabbit mAb (S-2295-7)Product Specification Host Rabbit Antigen beta Galactosidase GLB1 Synonyms Beta galactosidase; Acid beta galactosidase (Lactase); Elastin receptor 1; ELNR1 Immunogen Recombinant Protein Location Cytoplasm, Lysosome Accession P16278 Clone Number S 2295 7 Antibody Type Recombinant mAb Isotype IgG Application WB, IHC P Reactivity Hu Positive Sample SK BR 3, MCF7, Saos 2, U 87 MG, SH SY5Y Purification Protein A Concentration 0. 5 mg ml Conjugation
Product Specification
| Host | Rabbit |
| Antigen | beta Galactosidase/GLB1 |
| Synonyms | Beta-galactosidase; Acid beta-galactosidase (Lactase); Elastin receptor 1; ELNR1 |
| Immunogen | Recombinant Protein |
| Location | Cytoplasm, Lysosome |
| Accession | P16278 |
| Clone Number | S-2295-7 |
| Antibody Type | Recombinant mAb |
| Isotype | IgG |
| Application | WB, IHC-P |
| Reactivity | Hu |
| Positive Sample | SK-BR-3, MCF7, Saos-2, U-87 MG, SH-SY5Y |
| Purification | Protein A |
| Concentration | 0.5 mg/ml |
| Conjugation | Unconjugated |
| Physical Appearance | Liquid |
| Storage Buffer | PBS, 40% Glycerol, 0.05% BSA, 0.03% Proclin 300 |
| Stability & Storage | 12 months from date of receipt / reconstitution, -20 °C as supplied |
Dilution
| application | dilution | species |
| WB | 1:1000 | Hu |
| IHC-P | 1:500 | Hu, Ms, Rt |
Background
Beta-galactosidase (GLB1) is an essential lysosomal enzyme responsible for the hydrolysis of terminal galactose residues in glycolipids and glycoproteins. This enzyme plays a crucial role in the degradation and recycling of complex molecules within cells. Deficiencies or mutations in the GLB1 gene can lead to the accumulation of undigested substrates in lysosomes, resulting in severe genetic disorders such as GM1 gangliosidosis and Morquio B syndrome. These conditions are characterized by progressive neurological and skeletal abnormalities due to the impaired breakdown of specific glycosphingolipids and glycoproteins.
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Exchange/Return Notes
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