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GBA elisa kit :: Human Glucosylceramidase (GBA) ELISA Kit

Scan QR to view Datasheet Catalog #    MBS7200758
Typical Testing Data/Standard Curve (for reference only)
Unit / Price
48-Strip-Wells  /  $470 +1 FREE 8GB USB
96-Strip-Wells  /  $675 +1 FREE 8GB USB
5x96-Strip-Wells  /  $2,925 +3 FREE 8GB USB
10x96-Strip-Wells  /  $5,490 +6 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Glucosylceramidase (GBA), ELISA Kit

 Also Known As   

Human Glucosylceramidase (GBA) ELISA Kit

 Product Gene Name   

GBA elisa kit

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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  Manual Insert    Download PDF Manual View PDF Manual
 OMIM    gene 608013
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 Species Reactivity    Human
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 Specificity    This assay has high sensitivity and excellent specificity for detection of GBA. No significant cross-reactivity or interference between GBA and analogues was observed. NOTE: Limited by current skills and knowledge, it is impossible for us to complete the cross-reactivity detection between GBA and all the analogues, therefore, cross reaction may still exist in some cases.
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 Samples    Serum, plasma, cell culture supernatants, body fluid and tissue homogenate
 Assay Type    Quantitative Competitive
 Sensitivity    0.1 ng/mL
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 Preparation and Storage    Store all reagents at 2-8 degree C.
 Sample Preparation    We suggest pre-experimenting with neat (undiluted) samples, 1:2 or 1:4 dilutions. Please avoid diluting your samples more than 1:10 as it would exceed the dilution limit set for this kit. If the expected concentration of the target is beyond the detection range of the kit, please contact our technical support team
 Product Note    Our ELISA assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay.
 Other Notes    Small volumes of GBA elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
 Searchable Terms for GBA purchase    MBS7200758 is a ready-to-use microwell, strip plate Quantitative Competitive ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Glucosylceramidase (GBA) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing GBA, with an estimated sensitivity of 0.1 ng/mL. The ELISA analytical biochemical technique of the MBS7200758 kit is based on GBA antibody-GBA antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect GBA antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, GBA. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions such as Serum, plasma, cell culture supernatants, body fluid and tissue homogenate. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).
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Product Description specifically for GBA elisa kit

   Intended Uses: This GBA ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Human GBA.

Principle of the Assay: GBA ELISA kit applies the competitive enzyme immunoassay technique utilizing a monoclonal anti-GBA antibody and an GBA-HRP conjugate. The assay sample and buffer are incubated together with GBA-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the GBA concentration since GBA from samples and GBA-HRP conjugate compete for the anti-GBA antibody binding site. Since the number of sites is limited, as more sites are occupied by GBA from the sample, fewer sites are left to bind GBA-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The GBA concentration in each sample is interpolated from this standard curve.
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 Typical Testing Data/Standard Curve (for reference only) of GBA elisa kit    GBA elisa kit Typical Testing Data/Standard Curve (for reference only) image
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Sample Manual Insert of MBS7200758
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NCBI/Uniprot data below describe general gene information for GBA. It may not necessarily be applicable to this product.
 NCBI GI #    284807152
 NCBI GeneID    2629
 NCBI Accession #    NP_001165283.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_001171812.1 [Other Products]
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 UniProt Secondary Accession #    Q16545; Q4VX22; Q6I9R6; Q9UMJ8; A8K796; B7Z5G2; B7Z6S1; J3KQG4; J3KQK9 [Other Products]
 UniProt Related Accession #    P04062 [Other Products]
 Molecular Weight    54,471 Da
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 NCBI Official Full Name    glucosylceramidase isoform 3
 NCBI Official Synonym Full Names    glucosidase, beta, acid
 NCBI Official Symbol    GBA [Similar Products]
 NCBI Official Synonym Symbols   
GCB; GBA1; GLUC
[Similar Products]
 NCBI Protein Information    glucosylceramidase; beta-GC; alglucerase; imiglucerase; acid beta-glucosidase; beta-glucocerebrosidase; lysosomal glucocerebrosidase; glucosylceramidase-like protein; D-glucosyl-N-acylsphingosine glucohydrolase
 UniProt Protein Name    Glucosylceramidase
 UniProt Synonym Protein Names   
Acid beta-glucosidase; Alglucerase; Beta-glucocerebrosidase; Beta-GC; D-glucosyl-N-acylsphingosine glucohydrolase; Imiglucerase
 Protein Family    Glucosylceramidase
 UniProt Gene Name    GBA [Similar Products]
 UniProt Synonym Gene Names    GC; GLUC; Beta-GC [Similar Products]
 UniProt Entry Name    GLCM_HUMAN
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 NCBI Summary for GBA    This gene encodes a lysosomal membrane protein that cleaves the beta-glucosidic linkage of glycosylceramide, an intermediate in glycolipid metabolism. Mutations in this gene cause Gaucher disease, a lysosomal storage disease characterized by an accumulation of glucocerebrosides. A related pseudogene is approximately 12 kb downstream of this gene on chromosome 1. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2010]
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 UniProt Comments for GBA    GBA: Defects in GBA are the cause of Gaucher disease (GD); also known as glucocerebrosidase deficiency. GD is the most prevalent lysosomal storage disease, characterized by accumulation of glucosylceramide in the reticulo-endothelial system. Different clinical forms are recognized depending on the presence (neuronopathic forms) or absence of central nervous system involvement, severity and age of onset. Defects in GBA are the cause of Gaucher disease type 1 (GD1); also known as adult non-neuronopathic Gaucher disease. GD1 is characterized by hepatosplenomegaly with consequent anemia and thrombopenia, and bone involvement. The central nervous system is not involved. Defects in GBA are the cause of Gaucher disease type 2 (GD2); also known as acute neuronopathic Gaucher disease. GD2 is the most severe form and is universally progressive and fatal. It manifests soon after birth, with death generally occurring before patients reach two years of age. Defects in GBA are the cause of Gaucher disease type 3 (GD3); also known as subacute neuronopathic Gaucher disease. GD3 has central nervous manifestations. Defects in GBA are the cause of Gaucher disease type 3C (GD3C); also known as pseudo-Gaucher disease or Gaucher-like disease. Defects in GBA are the cause of Gaucher disease perinatal lethal (GDPL). It is a distinct form of Gaucher disease type 2, characterized by fetal onset. Hydrops fetalis, in utero fetal death and neonatal distress are prominent features. When hydrops is absent, neurologic involvement begins in the first week and leads to death within 3 months. Hepatosplenomegaly is a major sign, and is associated with ichthyosis, arthrogryposis, and facial dysmorphism. Perinatal lethal Gaucher disease is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders. Defects in GBA contribute to susceptibility to Parkinson disease (PARK). A complex neurodegenerative disorder characterized by bradykinesia, resting tremor, muscular rigidity and postural instability. Additional features are characteristic postural abnormalities, dysautonomia, dystonic cramps, and dementia. The pathology of Parkinson disease involves the loss of dopaminergic neurons in the substantia nigra and the presence of Lewy bodies (intraneuronal accumulations of aggregated proteins), in surviving neurons in various areas of the brain. The disease is progressive and usually manifests after the age of 50 years, although early-onset cases (before 50 years) are known. The majority of the cases are sporadic suggesting a multifactorial etiology based on environmental and genetic factors. However, some patients present with a positive family history for the disease. Familial forms of the disease usually begin at earlier ages and are associated with atypical clinical features. Belongs to the glycosyl hydrolase 30 family. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Hydrolase; EC 3.2.1.45; Glycan Metabolism - other glycan degradation; Lipid Metabolism - sphingolipid

Chromosomal Location of Human Ortholog: 1q21

Cellular Component: lysosomal lumen; lysosomal membrane

Molecular Function: protein binding; receptor binding; glucosylceramidase activity

Biological Process: glucosylceramide catabolic process; negative regulation of MAP kinase activity; sphingolipid metabolic process; skin morphogenesis; response to glucocorticoid stimulus; response to testosterone stimulus; positive regulation of protein amino acid dephosphorylation; regulation of water loss via skin; response to estrogen stimulus; negative regulation of inflammatory response; carbohydrate metabolic process; negative regulation of interleukin-6 production; sphingosine biosynthetic process; ceramide biosynthetic process; glycosphingolipid metabolic process; response to pH

Disease: Gaucher Disease, Type Ii; Dementia, Lewy Body; Parkinson Disease, Late-onset; Gaucher Disease, Perinatal Lethal; Gaucher Disease, Type Iiic; Gaucher Disease, Type Iii; Gaucher Disease, Type I
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 Research Articles on GBA    1. GBA mutations represent a major genetic risk factor for the development of Parkinson disease (PD); GBA-PD is clinically, pharmacologically, and pathologically virtually indistinguishable from sporadic PD [review]
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with GBA elisa kitPathways associated with GBA elisa kit
 Reference Product  PubMed Publications
 SMPD1 elisa kit  >2 publications with GBA and SMPD1
 UGCG elisa kit  >1 publications with GBA and UGCG
 GALC elisa kit  >1 publications with GBA and GALC
 Products by Pathway  Pathway Diagram
 Glycosphingolipid Metabolism Pathway antibodies  Glycosphingolipid Metabolism Pathway Diagram
 Lysosome Pathway antibodies  Lysosome Pathway Diagram
 Lysosome Pathway antibodies  Lysosome Pathway Diagram
 Metabolic Pathways antibodies  Metabolic Pathways Diagram
 Metabolism Pathway antibodies  Metabolism Pathway Diagram
 Metabolism Of Lipids And Lipoproteins Pathway antibodies  Metabolism Of Lipids And Lipoproteins Pathway Diagram
 Other Glycan Degradation Pathway antibodies  Other Glycan Degradation Pathway Diagram
 Other Glycan Degradation Pathway antibodies  Other Glycan Degradation Pathway Diagram
 Sphingolipid Metabolism Pathway antibodies  Sphingolipid Metabolism Pathway Diagram
 Sphingolipid Metabolism Pathway antibodies  Sphingolipid Metabolism Pathway Diagram
Diseases associated with GBA elisa kitOrgans/Tissues associated with GBA elisa kit
 Disease Name  Pubmed Publications
 Gaucher Disease Antibodies  >187 publications with GBA and Gaucher Disease
 Parkinson Disease Antibodies  >130 publications with GBA and Parkinson Disease
 Lewy Body Disease Antibodies  >20 publications with GBA and Lewy Body Disease
 Kidney Diseases Antibodies  >11 publications with GBA and Kidney Diseases
 Anemia Antibodies  >9 publications with GBA and Anemia
 Inflammation Antibodies  >8 publications with GBA and Inflammation
 Adenocarcinoma Antibodies  >7 publications with GBA and Adenocarcinoma
 Necrosis Antibodies  >7 publications with GBA and Necrosis
 Abnormalities, Drug-Induced Antibodies  >3 publications with GBA and Abnormalities, Drug-Induced
 Prostatic Diseases Antibodies  >3 publications with GBA and Prostatic Diseases
 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >106 publications with GBA and Brain
 Blood Antibodies  >98 publications with GBA and Blood
 Bone Antibodies  >34 publications with GBA and Bone
 Bone Marrow Antibodies  >20 publications with GBA and Bone Marrow
 Liver Antibodies  >17 publications with GBA and Liver
 Kidney Antibodies  >15 publications with GBA and Kidney
 Skin Antibodies  >15 publications with GBA and Skin
 Nerve Antibodies  >14 publications with GBA and Nerve
 Heart Antibodies  >13 publications with GBA and Heart
 Lung Antibodies  >12 publications with GBA and Lung
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