New Assay kits for Parkinson's Disease research
Approximately 5%–8% of people with Parkinson's disease have a genetic mutation in the Glucocerebrosidase (GBA) gene. It is suggested that such mutations contribute to the incidence of Parkinson's disease by limiting GBA's ability to clear out proteins like alpha-synuclein. SensoLyte® Glucocerebrosidase (GBA) Assay Kits were developed to evaluate GBA enzyme activity. This approach gives more accurate results than those obtained with kits that detect merely the total quantity of enzyme. (more about GBA).
Features & Benefits
We offer two (2) Fluorimetric based kits for GBA activity measurement and screening.
- Our Red GBA kit is a one-step homogenous assay, that is less prone to background interference, and is ideal for HTS and compound screens.
- Our Blue GBA kit is specifically validated for activator screening.
Wild type/Normal GBA (Gcase) gets transported from the ER
to the lysosome and breaks down Glucoceramide (GC) to Glucose
and ceramide. Ceramide regulates autophagy and helps clear out
Mutant GBA undergoes ER-associated degradation via proteasome and may
also cause a build-up of substrates including GC within the
lysosome thereby impairing autophagy leading to cell degeneration.
The autophagy lysosomal pathway (ALP) is an essential process, that is carried out by lysosomes, to routinely clear out senescent cells, including organelles, and toxic aggregated proteins. Glucocerebrosidase (GBA) is one of the many enzymes involved in ALP, and it hydrolyzes Glucocerebroside (also known as Glucoceramide), a normal component of the cell membrane, into glucose and ceramide.
When enzymatic activity of GBA is reduced due to gene mutation, glucocerebroside accumulates in the lysosomes causing a reduction in ceramide thereby downregulating autophagy, further impacting lysosomal dysfunction. This increasingly is regarded as a major pathogenic event in many neurodegenerative diseases, including Parkinson's disease (PD).
Mutations of the GBA1 gene are considered as crucial risk factors for the development of PD, with evidence pointing to the accumulation of misfolded alpha-synuclein proteins and lipids in the neuronal cells. In addition, it has been suggested that glucocerebroside, the substrate of GBA, may be involved in stabilization of the toxic oligomers of alpha-synuclein. Interestingly, it has been shown that even PD patients without GBA mutations demonstrate lower levels of GBA activity.