Name: Human GLA ELISA Matched Antibody Pair Set [ABP-Q-04-176]
SKU: ABP-Q-04-176
Price: 1120 USD

Human GLA ELISA Matched Antibody Pair Set [ABP-Q-04-176] (CAT#: ABP-Q-04-176)

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Human GLA antibody pair set for ELISA useCapture Antibody: Mouse anti-GLA monoclonal antibodyDetection Antibody: Mouse anti-GLA monoclonal antibody conjugated to HRP

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Type	Size	Price	Quantity
Antibody Pair Set-Package 1	5 × 96 tests	$1,120.00
Antibody Pair Set-Package 2	15 × 96 tests	$2,331.43

Datasheet

MSDS

Certificate of Analysis Lookup

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Lot Number

Antigen Detail
Target	GLA
Description	Alpha-galactosidase A, also known as Alpha-D-galactoside galactohydrolase, Alpha-D-galactosidase A, Melibiase and GLA, is a member of the glycosyl hydrolase 27 family. GLA is used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. Defects in GLA are the cause of Fabry disease (FD) which 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. Deficiency of GLA leads to the accumulation of glycosphingolipids in the vasculature leading to multiorgan pathology. In addition to well-described microvascular disease, deficiency of GLA is also characterized by premature macrovascular events such as stroke and possibly myocardial infarction.
Species Reactivity	Human

Capture Antibody
Capture Clonality	Monoclonal
Host Species	Mouse
Clone	IV01983
Purification	Affinity Chromatography
Buffer	Prior to lyophilization: PBS, pH 7.4
Application	ELISA

Detection Antibody
Det-Clonality	Monoclonal
Host Species	Mouse
Conjugation	HRP
Clone	RD10232
Purification	Affinity Chromatography
Buffer	Prior to lyophilization: PBS, containing 50 % HRP-Protector

General Product Property
Product Overview	Human GLA antibody pair set for ELISA use Capture Antibody: Mouse anti-GLA monoclonal antibody Detection Antibody: Mouse anti-GLA monoclonal antibody conjugated to HRP
Specificity	Quantitative determination of Human GLA
Range	156.25-10000 pg/mL
Research Area	Blood
Application	ELISA
Application Notes	Optimal dilutions should be determined experimentally by the user.
Form	Lyophilized
Storage	Avoid repeated freeze/thaw cycles. Store at 2-8°C for short term. Aliquot and store at -20°C for long term storage.
Size	1 Set
Sample Type	Serum; Supernatant; Urine; Cell lysate; Plasma; Tissue Homogenates
Usage Statement	Available for Research Use Only. Not for use in therapeutic or diagnostic applications.

Target Overview
Gene Symbol	GLA
Full Name	Galactosidase Alpha
Background	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.
Alternative Names	Alpha-Galactosidase A; Melibiase
Gene ID	2717
Uniprot ID	P06280

For Research Use Only.

Human GLA ELISA Matched Antibody Pair Set [ABP-Q-04-176] (CAT#: ABP-Q-04-176)

Antigen Detail

Capture Antibody

Detection Antibody

General Product Property

Target Overview