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Galactosylceramidase Recombinant Rabbit mAb (S-3402-17)

Galactosylceramidase Recombinant Rabbit mAb (S-3402-17)

Catalog Number: S0B6671 Application: WB, IHC-P Reactivity: Human,Mouse, Rat, Monkey Conjugation: Unconjugated Brand: Starter
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Regular price $100 USD
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Product Details

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.

Picture

Western Blot

WB result of Galactosylceramidase Recombinant Rabbit mAb
Primary antibody: Galactosylceramidase Recombinant Rabbit mAb at 1/1000 dilution
Lane 1: A549 whole cell lysate 20 µg
Lane 2: SH-SY5Y whole cell lysate 20 µg
Lane 3: A375 whole cell lysate 20 µg
Lane 4: HepG2 whole cell lysate 20 µg
Secondary antibody: Goat Anti- rabbit IgG, (H+L), HRP conjugated at 1/10000 dilution
Predicted MW: 77 kDa
Observed MW: 30, 50, 70, 170 kDa

WB result of Galactosylceramidase Recombinant Rabbit mAb
Primary antibody: Galactosylceramidase Recombinant Rabbit mAb at 1/1000 dilution
Lane 1: mouse brain lysate 20 µg
Secondary antibody: Goat Anti- rabbit IgG, (H+L), HRP conjugated at 1/10000 dilution
Predicted MW: 77 kDa
Observed MW: 30, 50, 65 kDa

WB result of Galactosylceramidase Recombinant Rabbit mAb
Primary antibody: Galactosylceramidase Recombinant Rabbit mAb at 1/1000 dilution
Lane 1: COS-7 whole cell lysate 20 µg
Secondary antibody: Goat Anti- rabbit IgG, (H+L), HRP conjugated at 1/10000 dilution
Predicted MW: 77 kDa
Observed MW: 30, 50 kDa

Immunohistochemistry

IHC shows positive staining in paraffin-embedded human cerebral cortex. Anti-Galactosylceramidase antibody was used at 1/1000 dilution, followed by a HRP Polymer for Mouse & Rabbit IgG (ready to use). Counterstained with hematoxylin. Heat mediated antigen retrieval with Tris/EDTA buffer pH9.0 was performed before commencing with IHC staining protocol.

IHC shows positive staining in paraffin-embedded human hepatocellular carcinoma. Anti-Galactosylceramidase antibody was used at 1/1000 dilution, followed by a HRP Polymer for Mouse & Rabbit IgG (ready to use). Counterstained with hematoxylin. Heat mediated antigen retrieval with Tris/EDTA buffer pH9.0 was performed before commencing with IHC staining protocol.

IHC shows positive staining in paraffin-embedded mouse cerebral cortex. Anti-Galactosylceramidase antibody was used at 1/1000 dilution, followed by a HRP Polymer for Mouse & Rabbit IgG (ready to use). Counterstained with hematoxylin. Heat mediated antigen retrieval with Tris/EDTA buffer pH9.0 was performed before commencing with IHC staining protocol.

IHC shows positive staining in paraffin-embedded rat cerebral cortex. Anti-Galactosylceramidase antibody was used at 1/1000 dilution, followed by a HRP Polymer for Mouse & Rabbit IgG (ready to use). Counterstained with hematoxylin. Heat mediated antigen retrieval with Tris/EDTA buffer pH9.0 was performed before commencing with IHC staining protocol.