- Phare
- Validé par KD/KO
Anticorps Polyclonal de lapin anti-Galc
Galc Polyclonal Antibody for WB, IHC, IF-P, IP, Indirect ELISA
Hôte / Isotype
Lapin / IgG
Réactivité testée
Humain, rat, souris
Applications
WB, IHC, IF-P, IP, Indirect ELISA
Conjugaison
Non conjugué
N° de cat : 11991-1-PBS
Synonymes
Galerie de données de validation
Informations sur le produit
11991-1-PBS cible Galc dans les applications de WB, IHC, IF-P, IP, Indirect ELISA et montre une réactivité avec des échantillons Humain, rat, souris
Réactivité | Humain, rat, souris |
Hôte / Isotype | Lapin / IgG |
Clonalité | Polyclonal |
Type | Anticorps |
Immunogène | Galc Protéine recombinante Ag3914 |
Nom complet | galactosylceramidase |
Masse moléculaire calculée | 77 kDa |
Poids moléculaire observé | 80 kDa, 30 kDa, 50 kDa |
Numéro d’acquisition GenBank | BC086671 |
Symbole du gène | Galc |
Identification du gène (NCBI) | 14420 |
Conjugaison | Non conjugué |
Forme | Liquide |
Méthode de purification | Purification par affinité contre l'antigène |
Tampon de stockage | PBS only |
Conditions de stockage | Store at -80°C. 20ul contiennent 0,1% de BSA. |
Informations générales
The GALC antibody targets the liposomal enzyme Galactosylceramidase (GALC), which belongs to the glycosyl hydrolase 59 family. It hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. It is primarily found in the brain and kidneys where galactolipids are hydrolyzed (PMID:8634707). Deficiencies of GALC are primarily associated with the autosomal recessive Krabbe's disease. This disease is characterized by developmental delay caused by apoptosis of myelin-forming cells. GALC is responsible for hydrolyzing galactosylceramide, a cerebroside that is an important component of myelin. A deficiency in GALC causes loss of myelin to nerve cells, resulting in delayed nerve transmissions. Krabbe's disease has varying degrees of severity due to a large number of different genetic mutations in the gene. The GALC antibody can be used to detect the deletions in the GALC gene and functions of the enzyme (PMID:20886637). Normal GALC mRNA encodes the 80 kDa precursor, which is processed into 50 and 30 kDa subunits (PMID: 26865610).