What does beta-galactosidase assay do?

What does beta-galactosidase assay do?

β-Galactosidase converts the colorless ONPG substrate into galactose and the chromophore o-nitrophenol, yielding a bright yellow solution. The β-Galactosidase Assay Kit is excellent for determining enzyme activity in lysates from cells transfected with a β-galactosidase expression construct.

What is the primary function of β-Galactosidase?

β-Galactosidase is an intracellular enzyme that is an essential part of the cellular metabolism of galactosides like lactose. It cleaves (separates) large substrate molecules into smaller ones by breaking the glycosidic bond.

What is the function of the beta-galactosidase protein?

β-Galactosidase (GLB1) catalyzes the degradation of galactosylceramide to galactose and ceramide within the lysosome. It also displays activity against galactosylsphingosine and lactosylceramide.

How is beta-galactosidase activity measured?

Beta-galactosidase, encoded by the lacZ gene in E. coli, can cleave lactose and structurally related compounds to galactose and glucose or structurally related products. Its activity can be measured using an artificial substrate, o-nitrophenyl-beta-D-galactopyranoside (ONPG).

What is beta-galactosidase made up of?

Beta-galactosidase consists of four chains, each with 1023 amino acids (blue), that form four active sites. The substrate/product allolactose (pink and white) can be seen here in two of these active sites. This structure is based on a high resolution (1.5 Angstrom) x-ray crystallographic study.

What is beta-galactosidase deficiency?

GM1 gangliosidosis, also called beta-galactosidase-1 deficiency, is a genetic disorder that progressively destroys nerve cells in the brain and spinal cord.

How is beta-galactosidase made?

The presence of lactose results in the synthesis of allolactose which binds to the lac repressor and reduces its affinity for the lac operon. This in turn allows the synthesis of β-galactosidase, the product of the lacZ gene.

Where is beta-galactosidase found?

lysosomes
The GLB1 gene provides instructions for producing an enzyme called beta-galactosidase (β-galactosidase). This enzyme is located in lysosomes, which are compartments within cells that break down and recycle different types of molecules.

Which product is made from immobilized beta-galactosidase enzyme?

GOS can be readily manufactured by enzymatic transgalactosylation of β-galactosidase from whey lactose, which is available in abundance as a by-product of cheese industry. Thus, the application of β-galactosidase in the hydrolysis of lactose in dairy industry has attracted the attention of researchers.

Which disease occurs as result of beta-galactosidase deficiency?

Beta-galactosidase deficiency is associated with three distinct autosomal recessive lysosomal storage disorders: GM1 gangliosidosis (GM1), mucopolysaccharidosis type IVB (MPS IVB), and galactosialidosis. GM1 and MPS IVB are referred to as GLB1-related disorders as they are the result of biallelic mutations in GLB1.

What would happen with GM1 in someone with a galactosidase deficiency?