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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
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-Zugangsnummer | BC086671 |
Gene symbol | Galc |
Gene ID (NCBI) | 14420 |
Konjugation | Unkonjugiert |
Form | Liquid |
Reinigungsmethode | Antigen-Affinitätsreinigung |
Lagerungspuffer | PBS only |
Lagerungsbedingungen | Store 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).