Glucosylceramidase ELISA Kit (GBA)
INTENDED USE
Human glucosylceramidase ELISA kit can measure concentrations of GBA (glucosylceramidase, beta-GC, GLUC, imiglucerase, acid beta-glucosidase, algucerase, GC) present is plasma, serum, biological fluids or tissue homogenate samples.
INTRODUCTION
GBA, or glucosylceramidase, is a lysosomal enzyme involved in the breakdown of glycosphingolipids. By cleaving glucose groups from lipid substrates, it helps recycle cellular components and maintain lipid homeostasis. Inherited mutations disrupting GBA enzyme function lead to glycolipid accumulation and corresponding nervous system pathology known as Gaucher disease. Elucidating the mechanisms linking diminished GBA activity to cell damage and disease holds therapeutic promise.
GBA breaks down glycolipids like glucocerebroside, allowing reusable constituent parts to exit lysosomes safely after digestion. When it is deficient, substrate accumulation damages cells through lipotoxic lysosomal dysfunction, disrupted calcium regulation, and possibly protein aggregation stress. Research also implicates connections between deficient GBA, glycolipid accumulation, and alpha-synuclein aggregation central to Parkinson’s disease neuropathology.
While severe GBA mutations cause neuron-impacting Gaucher disease, even subtle reductions in GBA-enzymatic activity associate with heightened Parkinson’s disease risk. This link reflects the importance of functional GBA for managing alpha-synuclein levels. When lysosomal digestion of old or damaged alpha-synuclein goes awry due to inadequate GBA, toxic oligomeric protein aggregates accumulate, driving neurodegeneration. Boosting GBA-activity appears protective against alpha-synuclein’s harmful effects.
Understanding connections between diminished GBA function, glycolipid processing deficits, and both lysosomal as well as alpha-synuclein dependent neuronal damage pathways will enable strategic therapeutic development for Gaucher disease and synucleinopathies like Parkinson’s. Small molecule chaperones assisting proper GBA-protein folding have shown promise by increasing lysosomal GBA levels. Alternatively, lowering upstream substrates through production blockade or alternative clearance mechanisms could also alleviate toxicity from substrate excess stemming from poor GBA function.
CONTENT
All reagents supplied need to be stored at 2 °C – 8 °C, unopened reagents will retain reactivity until expiration date. Do not use reagents beyond this date.
- One 96-Well Plate: Pre-coated with anti- GBA.
- Standards: Lyophilized recombinant.
- Sample/Standard Dilution Buffer.
- Biotinylated-labelled Antibody.
- Antibody Dilution Buffer.
- HRP-Streptavidin Conjugate (SABC).
- SABC Dilution Buffer.
- TMB Substrate.
- Wash Buffer (25x).
- Plate Sealer.
- Product Instructions.
TYPICAL RESULTS
For this GBA ELISA kit, it is recommended that a standard curve is generated for each assay carried out.
Standard Curve: 0, 0.312, 0.625, 1.25, 2.5, 5, 10, 20 ng/ml.
Reactivity: Human
Sensitivity: 0.188 ng/ml
Range: 0.313 – 20 ng/ml
Principle: Sandwich, Double Antibody
Application: Research Use Only.
ASSAY CHARACTERISTICS
– Specificity: Highly specific for GBA, no cross reactivity or interference between GBA and analogues was detected.
– Recovery: Serum (87 – 105%), EDTA Plasma (94 – 105%), Heparin Plasma (90 – 104%).
– Linearity: Serum (85 – 99%), EDTA Plasma (82 – 97%), Heparin Plasma (80 – 98%).
– Precison Intra-Assay: CV < 8%
– Precison Inter-Assay: CV < 10%
REFERENCES
- GBA Variants and Parkinson Disease: Mechanisms and Treatments. Cells. (2022) 11 (8): 1261. Smith L. and Schapira A.H.V.
- Gaucher disease: mutation and polymorphism spectrum in the glucocerebrosidase gene (GBA). Hum Mutat. (2008) 29 (5): 567-83. Hruska K.S., et al.
- GBA-Associated Parkinson’s Disease and Other Synucleinopathies. Curr Neurol Neurosci Rep. (2018) 18 (8): 44. Gan-Or Z., et al.
- The Clinical Profile of GBA-Related Lewy Body Disorders. JAMA Neurol. (2016) 73 (12): 1403-1404. Zabetian C.P.
ADDITIONAL INFORMATION
- Full Name: Glucosylceramidase ELISA Kit (GBA)
- Reactivity: Human
- Sample Type: Plasma, Tissue Homogenates, Serum, Biological Fluids
- Sensitivity: 0.188 ng/ml
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