Abcam, ab314825, Human Galactosidase alpha ELISA Kit

Size: 1 x 96Tests
Human Galactosidase alpha ELISA Kit is a sandwich ELISA designed to quantify Human Galactosidase alpha with a sensitivity of 45 pg/mL. - Colorimetric sandwich ELISA - 450 nm readout - works on any plate reader - Wide dynamic range - quantifies 45 - 10000 pg/mL
Key facts
Detection method:Colorimetric,
Sample types:Serum, Plasma, Cell culture supernatant,
Reacts with:Human,
Assay type:Sandwich (quantitative),
Sensitivity:= 45 pg/mL,
Range:45 - 10000 pg/mL,
Assay Platform:Pre-coated microplate (12 x 8 well strips)

Product details:
Human Galactosidase alpha ELISA Kit ab314825 is a sandwich ELISA to measure Human Galactosidase alpha in serum, plasma, cell culture supernatant with a sensitivity of 45 pg/ml.
How the assay works
This assay employs an antibody specific for human Galactosidase alpha coated on a 96-well plate.
Assay Specificity
Our ELISA kits are rigorously validated to ensure the highest level of consistency and reproducibility. Please check the protocol booklet for more details
Human Galactosidase alpha ELISA Kit ab314825 protocol summary
1. Add standard or sample to each well used. Incubate at room temperature
2. Wash and add prepared biotinylated antibody to each well. Incubate at room temperature.
3. Wash and add prepared streptavidin solution. Incubate at room temperature.
4. Add TMB Development Solution to each well. Incubate at room temperature.
5. Add Stop Solution to each well. Read at 450nm immediately.

Properties and Storage Information:
Shipped at conditions-Blue Ice, Appropriate short-term storage conditions--20°C, Appropriate long-term storage conditions--20°C, Storage information--20°C

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
Alpha-galactosidase also known as alpha-D-galactosidase or A-galactosidase is an enzyme that catalyzes the hydrolysis of terminal alpha-galactosyl moieties from glycolipids and glycoproteins. It has a molecular mass of approximately 50000 Daltons. This enzyme is expressed in various tissues including the liver heart and kidneys. It functions by efficiently removing alpha-galactose residues from target molecules a process important for maintaining cellular function.
Biological function summary
The role of alpha-galactosidase involves the breakdown of complex carbohydrates particularly those containing alpha-galactosidic bonds. It does not operate as part of a complex but rather functions independently to cleave these specific bonds in glycolipids and glycoproteins. This activity is critical for degrading langerin and other gangliosides preventing the accumulation of these molecules in the lysosomes. By continuously performing this function alpha-galactosidase maintains glycosphingolipid homeostasis ensuring efficient cellular metabolism.
Pathways
The involvement of alpha-galactosidase in metabolic and lysosomal catabolic pathways is significant. One critical pathway is the glycolipid metabolism pathway where it acts alongside related enzymes such as beta-galactosidase. It also participates in the lysosomal degradation pathway working in conjunction with other lysosomal enzymes to prevent substrate buildup. The interaction with enzymes like lysosomal hydrolases ensures the proper breakdown of complex molecules into simpler ones that cells can utilize or excrete.
Defects in the alpha-galactosidase enzyme lead to the lysosomal storage disorder known as Fabry disease. This genetic disorder causes the accumulation of globotriaosylceramide due to deficient alpha-galactosidase activity. Another disorder associated with malfunctioning alpha-galactosidase is cardiac complications due to tissue storage of glycolipids. In both cases the lack of enzyme activity disrupts cellular and tissue functions linking alpha-galactosidase to clinical symptoms seen in these disorders.