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GLA Antibody - N-terminal region : FITC (ARP54296_P050-FITC)

Product Specifications

Gene Name

Galactosidase, alpha

Gene Aliases

GALA

Gene ID

2717

Swiss Prot

P06280

Accession Number

NP_000160

Host

Rabbit

Reactivity

Human, Mouse, Rat, Cow, Dog, Goat, Guinea Pig, Horse, Rabbit, Zebrafish

Immunogen

The immunogen is a synthetic peptide directed towards the N terminal region of human GLA

Target

GLA is 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.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. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.

Partner Proteins

ZNF622; CNDP2; MAT2B; EIF4H; PGD; GNS; GBP2; G6PD; EIF5; CAPN1; ALDH7A1; TERT; UBC; FBXO6; OTUD4; GLA

Clonality

Polyclonal

Conjugation

FITC: Fluorescein Isothiocyanate

Type

Polyclonal Antibody

Applications

WB

Purification

Affinity Purified

Concentration

0.5 mg/ml

Homology

Cow: 93%; Dog: 100%; Goat: 86%; Guinea Pig: 100%; Horse: 86%; Human: 100%; Mouse: 100%; Rabbit: 93%; Rat: 100%; Zebrafish: 93%

Format

Liquid. Purified antibody supplied in 1x PBS buffer.

Reconstitution

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

Molecular Weight

45kDa

References & Citations

Kretz, K.A., (2008) Mol. Genet. Metab. 93 (3), 331-340

Shipping Conditions

Wet Ice

Product Datasheet

Anti-GLA (ARP54296_P050-FITC) antibody

Protein Length

429

NCBI Gene Symbol

GLA

NCBI GB Accession Number

GLA

Host or Source

Rabbit

Protein Name

Alpha-galactosidase A

Nucleotide Accession Number

NM_000169

Peptide Sequence

PQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFGYYDIDAQTF

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