GALC Antikörper (N-Term)

Produktnummer ABIN7879603

Der Kaninchen Polyklonal anti-GALC Antikörper (ABIN7879603) detektiert spezifisch GALC in WB. Dieser Antikörper reagiert spezifisch mit Proben aus Human, Ratte und Maus.

Kurzübersicht für GALC Antikörper (N-Term) (ABIN7879603)

Target: GALC (Galactosylceramidase (GALC))

Reaktivität: Human, Ratte, Maus

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GALC Antikörper ist unkonjugiert

Applikation: Western Blotting (WB)

Produktdetails anti-GALC Antikörper

Bindungsspezifität: N-Term

Verwendungszweck: GALC Antibody / Galactocerebrosidase

Aufreinigung: 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.

Isotyp: IgG

Anwendungsinformationen

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

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Rekonstitution: 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.

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: 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.

Details zu GALC

Target: GALC (Galactosylceramidase (GALC))

Andere Bezeichnung: GALC

Hintergrund: 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