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Home >> Antibodies >> GALC Antibody / Galactocerebrosidase Antibody

GALC Antibody / Galactocerebrosidase Antibody (FY13064)

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Image FY13064 Adding 0.2 ml of distilled water will yield a concentration of 500 ug/ml 100 ug 449
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GALC Antibody Human Neuroblastoma and Rodent Brain WB. Western blot analysis of GALC expression in multiple human cell and rodent tissue lysates. Lane 1: human U-373 whole cell lysate. Lane 2: human U-251 whole cell lysate. Lane 3: human SH-SY5Y whole cell lysate. Lane 4: human HEL whole cell lysate. Lane 5: rat brain tissue lysate. Lane 6: mouse brain tissue lysate. A single major band is detected at approximately 100 kDa in all samples using GALC antibody, consistent with the well-characterized N-glycosylated precursor form of Galactocerebrosidase. Although the predicted molecular weight of GALC is approximately 77 kDa, the mature enzyme is synthesized as a heavily glycosylated precursor of approximately 100 kDa that is subsequently processed into smaller lysosomal subunits. This result demonstrates that the GALC Antibody / Galactocerebrosidase Antibody is suitable for Western blot detection of endogenous GALC in human, rat, and mouse samples.
GALC Antibody Mouse Brain IHC. Immunohistochemical staining of FFPE mouse brain tissue using GALC antibody at 2 ug/ml following heat-induced epitope retrieval in pH8 EDTA buffer. Detection was performed using an HRP-conjugated secondary antibody with DAB chromogen. GALC immunoreactivity is observed throughout the brain parenchyma with predominantly cytoplasmic staining in neural cells, consistent with the lysosomal localization of Galactocerebrosidase, an essential enzyme involved in galactolipid metabolism and myelin maintenance. This result demonstrates that the GALC Antibody / Galactocerebrosidase Antibody is suitable for immunohistochemical detection of endogenous GALC in formalin-fixed, paraffin-embedded mouse tissue.
Species Reactivity Human, Mouse, Rat
Format Lyophilized
Host Rabbit
Clonality Polyclonal (rabbit origin)
Isotype Rabbit IgG
Purity Immunogen affinity purified
Buffer Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.
UniProt P54803
Applications Western Blot : 0.25-0.5ug/ml
Immunohistochemistry (FFPE) : 2-5ug/ml
Limitations This GALC Antibody / Galactocerebrosidase Antibody is available for research use only.
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Description

GALC Antibody / Galactocerebrosidase Antibody detects galactocerebrosidase, a lysosomal enzyme that catalyzes the hydrolysis of galactosylceramide and psychosine, both critical for normal myelin turnover. The UniProt recommended name is Galactocerebrosidase (GALC). This enzyme removes the galactose moiety from specific sphingolipids, supporting lipid recycling and the stability of central and peripheral nervous system myelin.

Functionally, GALC antibody recognizes a precursor glycoprotein that is proteolytically processed into 50 kDa and 30 kDa subunits forming the active heterodimeric enzyme within lysosomes. Galactocerebrosidase is essential for degrading galactolipids, and mutations in the GALC gene lead to Krabbe disease (globoid cell leukodystrophy), characterized by psychosine accumulation, oligodendrocyte death, and severe demyelination. The enzyme's deficiency disrupts lipid metabolism, resulting in neuroinflammation and white matter loss.

The GALC gene, located on chromosome 14q31.3, encodes a lysosomal hydrolase expressed in oligodendrocytes, Schwann cells, and various other tissues involved in lipid degradation. The protein's N-linked glycosylation and mannose-6-phosphate tagging ensure proper lysosomal trafficking. GALC activity is tightly regulated by substrate availability and cellular differentiation state, making it a key player in neuronal lipid homeostasis.

Pathologically, GALC dysfunction is the molecular hallmark of Krabbe disease, but altered expression has also been linked to other neurodegenerative and demyelinating disorders. Research using GALC antibody helps elucidate disease mechanisms and assess therapeutic strategies such as enzyme replacement or gene therapy. Immunodetection of GALC in cells or tissues serves as a biomarker for lysosomal integrity and treatment response.

GALC Antibody / Galactocerebrosidase Antibody is suitable for western blotting and immunohistochemistry to visualize lysosomal distribution and measure enzyme levels in normal and pathological tissues. It supports investigations into lysosomal storage disorders, lipid catabolism, and neuronal repair. NSJ Bioreagents provides GALC antibody reagents validated for lysosomal enzyme and myelin biology research.

Structurally, galactocerebrosidase contains a (beta/alpha)8-barrel catalytic core typical of glycosidases. Disease-causing mutations cluster around active-site residues or disrupt domain folding, leading to reduced stability or mislocalization. Studies using this antibody contribute to understanding GALC's enzymology, trafficking, and role in myelin preservation.

Explore our Neuroscience Antibodies page to discover validated antibodies for GALC and other proteins involved in lysosomal function, myelin maintenance, sphingolipid metabolism, neurodevelopment, and neurodegenerative disease research.

Application Notes

Optimal dilution of the GALC Antibody / Galactocerebrosidase Antibody should be determined by the researcher.

Immunogen

A synthetic peptide corresponding to a sequence at the N-terminus of human GALC was used as the immunogen for the GALC antibody.

Storage

After reconstitution, the GALC antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.

Alternate Names

GALC antibody, Galactocerebrosidase antibody, Galactosylceramidase antibody, Galactosylceramide Beta Galactosidase antibody, Beta Galactocerebrosidase antibody, Galactosylceramide Galactohydrolase antibody, Krabbe Disease Protein antibody, Psychosine Hydrolase antibody

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