Rabbit anti-Human GLA Polyclonal Antibody | anti-GLA antibody

GLA, NT (Alpha-galactosidase A, Alpha-D-galactoside Galactohydrolase, Alpha-D-galactosidase A, Agalsidase, Melibiase) (MaxLight 650)

Cat. Num. AAA6477815

• Gene Names: GLA; GALA
• Reactivity: Human
• Applications: Immunohistochemistry, Flow Cytometry, Functional Assay, Immunocytochemistry, Immunofluorescence, Western Blot
• Purity: Purified by Protein A/G Affinity Chromatography.
• Synonyms: GLA; Polyclonal Antibody; NT (Alpha-galactosidase A; Alpha-D-galactoside Galactohydrolase; Alpha-D-galactosidase A; Agalsidase; Melibiase) (MaxLight 650); EC=3.2.1.22; anti-GLA antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human GLA.
• Purity/Purification: Purified by Protein A/G Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight650.

• Applicable Applications for anti-GLA antibody: Immunohistochemistry (IHC) Paraffin, FLISA, Flow Cytometry (FC/FACS), Immunocytochemistry (ICC), Immunofluorescence (IF), Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 83-112 in the N-terminal region of human GLA, UniPot Accession #P06280.
• Conjugate: MaxLight650
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight650 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western Blot analysis of HeLa cell line lysates (35ug/lane) usingMBS6010695.)

Immunohistochemistry (IHC)

(Immunohistochemistry analysis of formalin fixed and paraffin embedded human lung carcinoma usingMBS6010695. Peroxidase-conjugated secondary antibody and DAB chromogen were used. )

Immunofluorescence (IF)

(Confocal immunofluorescent analysis of HeLa cells usingMBS6010695. Alexa Fluor 488-conjugated goat anti-rabbit lgG (green) was used as secondary antibody. DAPI was used to stain the cell nucleus (blue).)

Flow Cytometry (FC/FACS)

(Flow Cytometric analysis of HepG2 cells using MBS6010695 (right histogram) compared to a negative control cell (left histogram). FITC-conjugated goat-anti-rabbit secondary antibodies used for the analysis.)

Product Categories/Family for anti-GLA antibody

Antibodies; Enzymes; Glycosidase

References

1. Mignani, R., Kidney Int. 75(10), 1115-1116 (2009). 2. Ioannou, Y.A., Biochem. J. 332(PT 3), 789-797 (1998).

NCBI and Uniprot Product Information

• NCBI GI #: 4504009
• NCBI GeneID: 2717
• NCBI Accession #: NP_000160
• NCBI GenBank Nucleotide #: NM_000169.2
• UniProt Accession #: P06280; Q6LER7
• Molecular Weight: 46.4kDa (406aa) 40-57KDa (SDS-PAGE under reducing conditions.)
• NCBI Official Full Name: alpha-galactosidase A
• NCBI Official Synonym Full Names: galactosidase alpha
• NCBI Official Symbol: GLA
• NCBI Official Synonym Symbols: GALA
• NCBI Protein Information: alpha-galactosidase A
• UniProt Protein Name: Alpha-galactosidase A
• Protein Family: Glacontryphan
• UniProt Gene Name: GLA
• UniProt Entry Name: AGAL_HUMAN

NCBI Description

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 Description

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; Lipid Metabolism - sphingolipid; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Hydrolase; EC 3.2.1.22; Lipid Metabolism - glycerolipid

Chromosomal Location of Human Ortholog: Xq22

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

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

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

Disease: Fabry Disease