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Galc Polyklonaler Antikörper

Galc Polyklonal Antikörper für WB, IHC, IF-P, IP, Indirect ELISA

Wirt / Isotyp

Kaninchen / IgG

Getestete Reaktivität

human, Maus, Ratte

Anwendung

WB, IHC, IF-P, IP, Indirect ELISA

Konjugation

Unkonjugiert

Kat-Nr. : 11991-1-PBS

Synonyme

GALCERase, Galactosylceramide beta-galactosidase, Galactocerebroside beta-galactosidase, Galactocerebrosidase, Gacy



Geprüfte Anwendungen

Produktinformation

11991-1-PBS bindet in WB, IHC, IF-P, IP, Indirect ELISA Galc und zeigt Reaktivität mit human, Maus, Ratten

Getestete Reaktivität human, Maus, Ratte
Wirt / Isotyp Kaninchen / IgG
Klonalität Polyklonal
Typ Antikörper
Immunogen Galc fusion protein Ag3914
Vollständiger Name galactosylceramidase
Berechnetes Molekulargewicht 77 kDa
Beobachtetes Molekulargewicht 80 kDa, 30 kDa, 50 kDa
GenBank-ZugangsnummerBC086671
Gene symbol Galc
Gene ID (NCBI) 14420
Konjugation Unkonjugiert
Form Liquid
Reinigungsmethode Antigen-Affinitätsreinigung
Lagerungspuffer PBS only
LagerungsbedingungenStore at -80°C. 20ul Größen enthalten 0,1% BSA.

Hintergrundinformationen

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).