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anti-GLa antibody :: Mouse anti-Human Galactosidase Alpha (GLa) Monoclonal Antibody

Scan QR to view Datasheet Catalog #    MBS2090810 anti-GLa antibody
Unit / Price
0.01 mg  /  $105 +1 FREE 8GB USB
0.02 mg  /  $110 +1 FREE 8GB USB
0.05 mg  /  $145 +1 FREE 8GB USB
0.1 mg  /  $185 +1 FREE 8GB USB
0.2 mg  /  $235 +1 FREE 8GB USB
1 mg  /  $540 +1 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   

Galactosidase Alpha (GLa), Monoclonal Antibody

 Also Known As   

Biotin-Linked Monoclonal Antibody to Galactosidase Alpha (GLa)

 Product Gene Name   

anti-GLa antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    X05790 mRNA
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 Clonality    Monoclonal
 Host    Mouse
 Species Reactivity    Human
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 Concentration    500ug/ml (lot specific)
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 Conjugation    Biotin
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 Other Notes    Small volumes of anti-GLa antibody 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.
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 Applications Tested/Suitable for anti-GLa antibody   

Western Blot (WB), Immunohistochemistry (IHC)

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NCBI/Uniprot data below describe general gene information for GLa. It may not necessarily be applicable to this product.
 NCBI GI #    957554387
 NCBI GeneID    2717
 NCBI Accession #    BAT62474.1 [Other Products]
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 UniProt Secondary Accession #    Q6LER7 [Other Products]
 UniProt Related Accession #    P06280 [Other Products]
 Molecular Weight    48,767 Da
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 NCBI Official Full Name    galactosidase, alpha, partial
 NCBI Official Synonym Full Names    galactosidase alpha
 NCBI Official Symbol    GLA [Similar Products]
 NCBI Official Synonym Symbols   
GALA
[Similar Products]
 NCBI Protein Information    alpha-galactosidase A
 UniProt Protein Name    Alpha-galactosidase A
 UniProt Synonym Protein Names   
Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Melibiase; INN: Agalsidase
 Protein Family    Glycerol facilitator-aquaporin
 UniProt Gene Name    GLA [Similar Products]
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 NCBI Summary for GLa    This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties. [provided by RefSeq, Jul 2008]
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 UniProt Comments for GLa    GLA: Defects in GLA are the cause of Fabry disease (FD). FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities. Belongs to the glycosyl hydrolase 27 family.

Protein type: Carbohydrate Metabolism - galactose; EC 3.2.1.22; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Hydrolase; Lipid Metabolism - glycerolipid; Lipid Metabolism - sphingolipid

Chromosomal Location of Human Ortholog: Xq22.1

Cellular Component: cytoplasm; extracellular region; Golgi apparatus; lysosomal lumen; lysosome

Molecular Function: alpha-galactosidase activity; catalytic activity; galactoside binding; hydrolase activity; protein binding; protein homodimerization activity; receptor binding

Biological Process: glycoside catabolic process; glycosphingolipid catabolic process; glycosphingolipid metabolic process; glycosylceramide catabolic process; negative regulation of nitric oxide biosynthetic process; negative regulation of nitric-oxide synthase activity; neutrophil degranulation; oligosaccharide metabolic process

Disease: Fabry Disease
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 Research Articles on GLa    1. Enzyme activities (acid alpha-glucosidase (GAA), galactocerebrosidase (GALC), glucocerebrosidase (GBA), alpha-galactosidase A (GLA), alpha-iduronidase (IDUA) and sphingomyeline phosphodiesterase-1 (SMPD-1)) were measured on ~43,000 de-identified dried blood spot (DBS) punches, and screen positive samples were submitted for DNA sequencing to obtain genotype confirmation of disease risk
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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 anti-GLa antibodyPathways associated with anti-GLa antibody
 Reference Product  PubMed Publications
 GAA antibody  >11 publications with GLa and GAA
 GALC antibody  >6 publications with GLa and GALC
 ARSA antibody  >5 publications with GLa and ARSA
 HEXA antibody  >3 publications with GLa and HEXA
 GLB1 antibody  >3 publications with GLa and GLB1
 HEXB antibody  >2 publications with GLa and HEXB
 GAL3ST1 antibody  >1 publications with GLa and GAL3ST1
 Products by Pathway  Pathway Diagram
 Galactose Metabolism Pathway antibodies  Galactose Metabolism Pathway Diagram
 Galactose Metabolism Pathway antibodies  Galactose Metabolism Pathway Diagram
 Glycerolipid Metabolism Pathway antibodies  Glycerolipid Metabolism Pathway Diagram
 Glycerolipid Metabolism Pathway antibodies  Glycerolipid Metabolism Pathway Diagram
 Glycosphingolipid Biosynthesis - Globo And Isoglobo Series Pathway antibodies  Glycosphingolipid Biosynthesis - Globo And Isoglobo Series Pathway Diagram
 Glycosphingolipid Metabolism Pathway antibodies  Glycosphingolipid Metabolism Pathway Diagram
 Immune System Pathway antibodies  Immune System Pathway Diagram
 Innate Immune System Pathway antibodies  Innate Immune System Pathway Diagram
 Lysosome Pathway antibodies  Lysosome Pathway Diagram
 Lysosome Pathway antibodies  Lysosome Pathway Diagram
Diseases associated with anti-GLa antibodyOrgans/Tissues associated with anti-GLa antibody
 Disease Name  Pubmed Publications
 Nervous System Diseases Antibodies  >1056 publications with GLa and Nervous System Diseases
 Heart Diseases Antibodies  >488 publications with GLa and Heart Diseases
 Inflammation Antibodies  >401 publications with GLa and Inflammation
 Fabry Disease Antibodies  >347 publications with GLa and Fabry Disease
 Disease Models, Animal Antibodies  >323 publications with GLa and Disease Models, Animal
 Kidney Diseases Antibodies  >315 publications with GLa and Kidney Diseases
 Hypertension Antibodies  >278 publications with GLa and Hypertension
 Carcinoma Antibodies  >204 publications with GLa and Carcinoma
 Pain Antibodies  >198 publications with GLa and Pain
 Necrosis Antibodies  >196 publications with GLa and Necrosis
 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >2415 publications with GLa and Blood
 Bone Antibodies  >1399 publications with GLa and Bone
 Vascular Antibodies  >907 publications with GLa and Vascular
 Heart Antibodies  >651 publications with GLa and Heart
 Brain Antibodies  >557 publications with GLa and Brain
 Connective Tissue Antibodies  >542 publications with GLa and Connective Tissue
 Kidney Antibodies  >500 publications with GLa and Kidney
 Liver Antibodies  >302 publications with GLa and Liver
 Skin Antibodies  >217 publications with GLa and Skin
 Lung Antibodies  >167 publications with GLa and Lung
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