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

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Catalog # MBS2090810
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
anti-GLa antibody
Product Name

Galactosidase Alpha (GLa), Monoclonal Antibody

Also Known As

Biotin-Linked Monoclonal Antibody to Galactosidase Alpha (GLa)

Product Gene Name
Matching Pairs
Matching Pairs
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Immunogen Sequence Length
58
OMIM
X05790 mRNA
Clonality
Monoclonal
Host
Mouse
Species Reactivity
Human
Concentration
500ug/ml (lot specific)
Conjugation
Biotin
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.
Applications Tested/Suitable for anti-GLa antibody
Western Blot (WB), Immunohistochemistry (IHC)
NCBI/Uniprot data below describe general gene information for GLa. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
48,767 Da
NCBI Official Full Name
galactosidase, alpha, partial
NCBI Official Synonym Full Names
galactosidase alpha
NCBI Official Symbol
NCBI Official Synonym Symbols
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
UniProt Gene Name
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]
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
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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