127338-ML550
GLB1 (Beta-galactosidase, Acid beta-galactosidase, Lactase, Elastin Receptor 1, ELNR1) (MaxLight 550)
Size
£914.00
- SKU:
- 127338-ML550
- Purity:
- Purified by Protein A affinity chromatography.
- Storage Conditions:
- 4°C Do Not Freeze
- Supplier:
- United States Biological
- Host:
- Mouse
- Immunogen:
- Partial recombinant corresponding to aa578-677 from human GLB1 (NP_000395) with GST tag. MW of the GST tag alone is 26kD.
- Clone:
- 6E7
- Format:
- Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight™550.
- Extra Details:
- MaxLight™550 is a new Yellow-Green photostable dye conjugate comparable to Alexa Fluor™546, 555, DyLight™549 , Cy3™, TRITC and offers better labeling efficiency, brighter imaging and increased immunodetection. Absorbance (550nm); Emission (575nm); Extinction Coefficient 150,000. GLB1, a 60-76kD glycoprotein, is a lysosomal B-galactosidase that hydrolyzes the terminal B-galactose from ganglioside and keratan sulfate. Defects in this gene are the causes of lysosomal storage diseases for GM1-gangliosidosis and Morquio B syndrome. In GM1 gangliosidosis, GM1 ganglioside accumulates in the neurons of the central nervous system, because of the deficiency (0±3% of normal) of lysosomal B-galactosidase activity. GM1 gangliosidosis demonstrates varying degrees of clinical severity but is invariably fatal, and children with the most common and severe form of GM1 gangliosidosis usually die within 3 years of birth. Morquio B syndrome patients are neurologically normal, but display severe skeletal dysostosis multiplex because of an accumulation of keratan sulfate. More than 100 mutations have been identified for GLB1, which result in different residual activities of the mutant enzymes and a spectrum of symptoms in the two related diseases. In lysosome, the mature B-galactosidase protein associates with cathepsin A and neuraminidase 1 to form the lysosomal multienzyme complex. An alternative splicing at the RNA level of GLB1 results a catalytically inactive B-galactosidase (also called elastin-binding protein) that plays an important role in vascular development. Applications: Suitable for use in FLISA, Western Blot and Immunoprecipitation. Other applications not tested. Recommended Dilution: Optimal dilutions to be determined by the researcher. AA Sequence: KGQVWINGFNLGRYWPARGPQLTLFVPQHILMTSAPNTITVLELEWAPCSSDDPELCAVTFVDRPVIGSSVTYDHPSKPVEKRLMPPPPQKNKDSWLDHV Storage and Stability: Store product at 4°C in the dark. DO NOT FREEZE! Stable at 4°C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight™550 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap. Note: Applications are based on unconjugated antibody.
- Shipping Conditions:
- Blue Ice