GLA anticorps (AA 101-200) (Biotin)

N° du produit ABIN898243

Cet anticorps Lapin Polyclonal détecte spécifiquement GLA dans WB, ELISA, IHC (p) et IHC (fro). Il présente une réactivité envers Humain, Souris et Rat.

Aperçu rapide pour GLA anticorps (AA 101-200) (Biotin) (ABIN898243)

Antigène: GLA (Galactosidase, alpha (GLA))

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GLA est conjugé à/à la Biotin

Application: Western Blotting (WB), ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Détail du produit anti-GLA anticorps (Biotin)

Épitope: AA 101-200

Réactivité croisée: Humain, Souris, Rat

Homologie: Dog,Cow,Pig,Rabbit

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human Galactosidase alpha

Isotype: IgG

Information d'application

Indications d'application: WB 1:300-5000
IHC-P 1:200-400
IHC-F 1:100-500

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Stock: -20 °C

Stockage commentaire: Store at -20°C for 12 months.

Date de péremption: 12 months

Détails sur GLA

Antigène: GLA (Galactosidase, alpha (GLA))

Autre désignation: Galactosidase alpha

Sujet:

Synonyms: GALA, Alpha-galactosidase A, Alpha-D-galactosidase A, Alpha-D-galactoside galactohydrolase, Melibiase, GLA

Background: Galactosidase alpha is involved in the hydrolysis of terminal, non reducing alpha D galactose residues in alpha D galactosides, including galactose oligosaccharides, galactomannans and galactohydrolase. Defects in GLA are the cause of Fabry's disease (FD). FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. 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.

ID gène: 2717

UniProt: P06280

Pathways: SARS-CoV-2 Protein Interactome