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GM2AP elisa kit :: Rabbit GM2 Ganglioside Activator protein ELISA Kit

Scan QR to view Datasheet Catalog #    MBS723936 GM2AP elisa kit
Unit / Price
48-Strip-Wells  /  $470 +1 FREE 8GB USB
96-Strip-Wells  /  $675 +1 FREE 8GB USB
5x96-Strip-Wells  /  $2,850 +3 FREE 8GB USB
10x96-Strip-Wells  /  $5,340 +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   

GM2 Ganglioside Activator protein (GM2AP), ELISA Kit

 Also Known As   

Rabbit GM2 Ganglioside Activator protein ELISA Kit

 Product Gene Name   

GM2AP elisa kit

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Request for Manual Insert    Request Manual
 OMIM    613109
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 Species Reactivity    Rabbit
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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 technical support.
 Other Notes    Small volumes of GM2AP 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 GM2AP purchase    MBS723936 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the GM2 Ganglioside Activator protein (GM2AP) 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 GM2AP. The ELISA analytical biochemical technique of the MBS723936 kit is based on GM2AP antibody-GM2AP antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect GM2AP antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, GM2AP. Appropriate sample types may include undiluted human body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).
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 Product Categories/Family for GM2AP elisa kit    Signal Transduction
NCBI/Uniprot data below describe general gene information for GM2AP. It may not necessarily be applicable to this product.
 NCBI GI #    263190474
 NCBI GeneID    2760
 NCBI Accession #    NP_001161079.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_001167607.1 [Other Products]
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 UniProt Secondary Accession #    Q14426; Q14428; Q6LBL5; B2R699; D3DQH6 [Other Products]
 UniProt Related Accession #    P17900 [Other Products]
 Molecular Weight    20,838 Da
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 NCBI Official Full Name    ganglioside GM2 activator isoform 2
 NCBI Official Synonym Full Names    GM2 ganglioside activator
 NCBI Official Symbol    GM2A [Similar Products]
 NCBI Official Synonym Symbols   
SAP-3; GM2-AP
[Similar Products]
 NCBI Protein Information    ganglioside GM2 activator; shingolipid activator protein 3; sphingolipid activator protein 3; cerebroside sulfate activator protein
 UniProt Protein Name    Ganglioside GM2 activator
 UniProt Synonym Protein Names   
Cerebroside sulfate activator protein; GM2-AP; Sphingolipid activator protein 3; SAP-3
 UniProt Gene Name    GM2A [Similar Products]
 UniProt Synonym Gene Names    SAP-3 [Similar Products]
 UniProt Entry Name    SAP3_HUMAN
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 NCBI Summary for GM2AP    This gene encodes a small glycolipid transport protein which acts as a substrate specific co-factor for the lysosomal enzyme beta-hexosaminidase A. Beta-hexosaminidase A, together with GM2 ganglioside activator, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene result in GM2-gangliosidosis type AB or the AB variant of Tay-Sachs disease. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Nov 2009]
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 UniProt Comments for GM2AP    GM2A: The large binding pocket can accommodate several single chain phospholipids and fatty acids, GM2A also exhibits some calcium-independent phospholipase activity. Binds gangliosides and stimulates ganglioside GM2 degradation. It stimulates only the breakdown of ganglioside GM2 and glycolipid GA2 by beta-hexosaminidase A. It extracts single GM2 molecules from membranes and presents them in soluble form to beta- hexosaminidase A for cleavage of N-acetyl-D-galactosamine and conversion to GM3. Defects in GM2A are the cause of GM2-gangliosidosis type AB (GM2GAB); also known as Tay-Sachs disease AB variant. GM2-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2GAB is characterized by GM2 gangliosides accumulation in the presence of both hexosaminidase A and B.

Protein type: Mitochondrial; Activator

Chromosomal Location of Human Ortholog: 5q33.1

Cellular Component: lysosomal lumen; internal side of plasma membrane; apical cortex; mitochondrion; hydrogen:potassium-exchanging ATPase complex

Molecular Function: lipid transporter activity; beta-N-acetylgalactosaminidase activity; phospholipase activator activity

Biological Process: oligosaccharide catabolic process; sequestering of lipid; sphingolipid metabolic process; learning and/or memory; ganglioside catabolic process; positive regulation of hydrolase activity; glycosphingolipid metabolic process; neuromuscular process controlling balance; lipid transport

Disease: Gm2-gangliosidosis, Ab Variant
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 Research Articles on GM2AP    1. In vitro assays with the isolated H1 or H2 homodimers (beta-alpha hybrid construct of beta-hexosaminidase A subunits) confirmed that neither was capable of human GM2AP-dependent hydrolysis of GM2 ganglioside.
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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 GM2AP elisa kitPathways associated with GM2AP elisa kit
 Reference Product  PubMed Publications
 HEXB elisa kit  >9 publications with GM2AP and HEXB
 HEXA elisa kit  >9 publications with GM2AP and HEXA
 PSAP elisa kit  >1 publications with GM2AP and PSAP
 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
 Metabolism Pathway antibodies  Metabolism Pathway Diagram
 Metabolism Of Lipids And Lipoproteins Pathway antibodies  Metabolism Of Lipids And Lipoproteins Pathway Diagram
 Sphingolipid Metabolism Pathway antibodies  Sphingolipid Metabolism Pathway Diagram
Diseases associated with GM2AP elisa kitOrgans/Tissues associated with GM2AP elisa kit
 Disease Name  Pubmed Publications
 Nervous System Diseases Antibodies  >10 publications with GM2AP and Nervous System Diseases
 Brain Diseases Antibodies  >9 publications with GM2AP and Brain Diseases
 Tay-Sachs Disease, AB Variant Antibodies  >8 publications with GM2AP and Tay-Sachs Disease, AB Variant
 Disease Models, Animal Antibodies  >4 publications with GM2AP and Disease Models, Animal
 Kidney Diseases Antibodies  >3 publications with GM2AP and Kidney Diseases
 Breast Neoplasms Antibodies  >1 publications with GM2AP and Breast Neoplasms
 Atrophy Antibodies  >1 publications with GM2AP and Atrophy
 Organ/Tissue Name  Pubmed Publications
 Kidney Antibodies  >5 publications with GM2AP and Kidney
 Brain Antibodies  >4 publications with GM2AP and Brain
 Liver Antibodies  >3 publications with GM2AP and Liver
 Testis Antibodies  >2 publications with GM2AP and Testis
 Eye Antibodies  >1 publications with GM2AP and Eye
 Bone Marrow Antibodies  >1 publications with GM2AP and Bone Marrow
 Ovary Antibodies  >1 publications with GM2AP and Ovary
 Bone Antibodies  >1 publications with GM2AP and Bone
 Spleen Antibodies  >1 publications with GM2AP and Spleen
 Blood Antibodies  >1 publications with GM2AP and Blood
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