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Galc Polyklonaler Antikörper

Galc Polyklonal Antikörper für IF-P

Wirt / Isotyp

Kaninchen / IgG

Getestete Reaktivität

human, Maus, Ratte

Anwendung

IF-P

Konjugation

CoraLite® Plus 488 Fluorescent Dye

Kat-Nr. : CL488-11991

Synonyme

EC:3.2.1.46, Gacy, Galactocerebrosidase, Galactocerebroside beta-galactosidase, Galactosylceramide beta-galactosidase



Geprüfte Anwendungen

Erfolgreiche Detektion in IF-PMaushirngewebe

Empfohlene Verdünnung

AnwendungVerdünnung
Immunfluoreszenz (IF)-PIF-P : 1:50-1:500
It is recommended that this reagent should be titrated in each testing system to obtain optimal results.
Sample-dependent, check data in validation data gallery

Produktinformation

CL488-11991 bindet in IF-P 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 CoraLite® Plus 488 Fluorescent Dye
Excitation/Emission maxima wavelengths493 nm / 522 nm
Form Liquid
Reinigungsmethode Antigen-Affinitätsreinigung
Lagerungspuffer PBS with 50% glycerol, 0.05% Proclin300, 0.5% BSA
LagerungsbedingungenBei -20°C lagern. Vor Licht schützen. Nach dem Versand ein Jahr stabil. Aliquotieren ist bei -20oC Lagerung nicht notwendig. 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).

Protokolle

PRODUKTSPEZIFISCHE PROTOKOLLE
IF protocol for CL Plus 488 Galc antibody CL488-11991Protokoll herunterladen
STANDARD-PROTOKOLLE
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