SKU: 48298960823

Galactosylceramidase Recombinant Rabbit mAb (S-3402-17)

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Description

Galactosylceramidase Recombinant Rabbit mAb (S-3402-17)Product Specification Host Rabbit Antigen Galactosylceramidase Synonyms Galactocerebrosidase; GALCERase; Galactocerebroside beta galactosidase; Galactosylceramide beta galactosidase; Galc Immunogen Recombinant Protein Location Lysosome Accession P54818 Clone Number S 3402 17 Antibody Type Recombinant mAb Isotype IgG Application WB, IHC P Reactivity Hu, Ms, Rt, Mk Positive Sample A549, SH SY5Y, A375, HepG2, mouse brain, COS 7 Purification Protein A

Product Specification


Host Rabbit
Antigen Galactosylceramidase
Synonyms Galactocerebrosidase; GALCERase; Galactocerebroside beta-galactosidase; Galactosylceramide beta-galactosidase; Galc
Immunogen Recombinant Protein
Location Lysosome
Accession P54818
Clone Number S-3402-17
Antibody Type Recombinant mAb
Isotype IgG
Application WB, IHC-P
Reactivity Hu, Ms, Rt, Mk
Positive Sample A549, SH-SY5Y, A375, HepG2, mouse brain, COS-7
Purification Protein A
Concentration 0.5 mg/ml
Conjugation Unconjugated
Physical Appearance Liquid
Storage Buffer

PBS, 40% Glycerol, 0.05% BSA, 0.03% Proclin 300

Stability & Storage

12 months from date of receipt / reconstitution, -20 °C as supplied

Dilution


application dilution species
WB 1:1000 Hu, Ms, Mk
IHC-P 1:1000 Hu, Ms, Rt

Background

Galactosylceramidase, also known as galactocerebrosidase (GALC), is a lysosomal enzyme encoded by the GALC gene on chromosome 14q31.3 that catalyzes the hydrolytic cleavage of galactose from galactosylceramide and other galactolipids, playing a crucial role in myelin maintenance and turnover; deficiency in this enzyme leads to the accumulation of toxic metabolites, particularly psychosine, resulting in Krabbe disease (globoid cell leukodystrophy), a severe neurodegenerative disorder characterized by demyelination, multinucleated globoid cells in the white matter, and progressive loss of motor and cognitive functions, with early-onset forms typically manifesting within the first six months of life and proving fatal within a few years.

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SKU: 48298960823

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