GALC antibody (N-Term)

Catalog No. ABIN7879603

The Rabbit Polyclonal anti-GALC antibody (ABIN7879603) specifically detects GALC in WB. The antibody is reactive with Human, Rat and Mouse samples.

Quick Overview for GALC antibody (N-Term) (ABIN7879603)

Target: GALC (Galactosylceramidase (GALC))

Reactivity: Human, Rat, Mouse

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GALC antibody is un-conjugated

Application: Western Blotting (WB)

Product Details anti-GALC Antibody

Binding Specificity: N-Term

Purpose: GALC Antibody / Galactocerebrosidase

Purification: Immunogen affinity purified

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

Isotype: IgG

Application Details

Application Notes: Optimal dilution of the GALC antibody should be determined by the researcher.

Restrictions: For Research Use only

Handling

Format: Lyophilized

Reconstitution: Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Storage: 4 °C,-20 °C

Storage Comment: 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.

Target Details for GALC

Target: GALC (Galactosylceramidase (GALC))

Alternative Name: GALC

Background: GALC 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 enzymei 1/2s 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 proteini 1/2s 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 is suitable for western blotting, immunocytochemistry, 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.

UniProt: P54803