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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
Geprüfte Anwendungen
Erfolgreiche Detektion in IF-P | Maushirngewebe |
Empfohlene Verdünnung
Anwendung | Verdünnung |
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Immunfluoreszenz (IF)-P | IF-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-Zugangsnummer | BC086671 |
Gene symbol | Galc |
Gene ID (NCBI) | 14420 |
Konjugation | CoraLite® Plus 488 Fluorescent Dye |
Excitation/Emission maxima wavelengths | 493 nm / 522 nm |
Form | Liquid |
Reinigungsmethode | Antigen-Affinitätsreinigung |
Lagerungspuffer | PBS with 50% glycerol, 0.05% Proclin300, 0.5% BSA |
Lagerungsbedingungen | Bei -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 | |
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IF protocol for CL Plus 488 Galc antibody CL488-11991 | Protokoll herunterladen |
STANDARD-PROTOKOLLE | |
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Klicken Sie hier, um unsere Standardprotokolle anzuzeigen |