{"product_id":"galc-antibody-galactocerebrosidase-bha17135966","title":"GALC Antibody \/ Galactocerebrosidase","description":"\u003ch2\u003eOverview\u003c\/h2\u003e\u003cp\u003eGALC Antibody \/ Galactocerebrosidase is a anti-GALC Rabbit antibody Polyclonal (rabbit origin) supplied in Lyophilized format. Recommended for workflows such as Western blot (WB) with listed reactivity in Human, Mouse, Rat.\u003c\/p\u003e\u003ch2\u003eKey elements and design rationale\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003e\n\u003cstrong\u003eTarget:\u003c\/strong\u003e GALC\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eAntibody details:\u003c\/strong\u003e Rabbit, Polyclonal (rabbit origin), isotype Rabbit IgG\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eFormat:\u003c\/strong\u003e Lyophilized\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eApplications (as listed):\u003c\/strong\u003e WB\u003c\/li\u003e\n\u003c\/ul\u003e\u003ch2\u003eBiological background\u003c\/h2\u003e\u003cdiv\u003eGALC 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.\u003cbr\u003e\u003cbr\u003eFunctionally, 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.\u003cbr\u003e\u003cbr\u003eThe 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.\u003cbr\u003e\u003cbr\u003ePathologically, 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.\u003cbr\u003e\u003cbr\u003eGALC 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. \u003cbr\u003e\u003cbr\u003eStructurally, 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.\u003c\/div\u003e\u003ch2\u003eResearch relevance and current trends\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003eConnecting protein-level changes to phenotype using orthogonal readouts (genetic perturbation, transcriptomics, imaging).\u003c\/li\u003e\n\u003cli\u003eConsidering isoforms and post-translational regulation when interpreting protein-level changes.\u003c\/li\u003e\n\u003cli\u003eComparing results across species and model systems with matched controls.\u003c\/li\u003e\n\u003c\/ul\u003e\u003ch2\u003eCommon research applications\u003c\/h2\u003e\u003cul\u003e\u003cli\u003e\n\u003cstrong\u003eWestern blotting:\u003c\/strong\u003e compare relative abundance and activation-state changes across conditions.\u003c\/li\u003e\u003c\/ul\u003e\u003cp\u003eInterpret changes in signal alongside appropriate controls and, when relevant, in parallel with total-protein or pathway readouts.\u003c\/p\u003e\u003ch2\u003eNotes for experimental interpretation\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003eSignal can reflect expression level, isoform composition, and post-translational state; interpret results in the context of your model system and stimuli.\u003c\/li\u003e\n\u003cli\u003eSpecies differences and sample matrices can influence epitope recognition; prioritize matched controls and orthogonal confirmation when feasible.\u003c\/li\u003e\n\u003c\/ul\u003e\u003cp\u003e\u003cstrong\u003eAntibody notes:\u003c\/strong\u003e Polyclonal antibodies recognize multiple epitopes, which can broaden the epitope footprint and may increase sensitivity in some contexts.\u003c\/p\u003e\u003c!-- Sources (internal): - UniProt search — UniProt — https:\/\/www.uniprot.org\/uniprotkb?query=GALC - NCBI Gene search — NCBI — https:\/\/www.ncbi.nlm.nih.gov\/gene\/?term=GALC - Ensembl search — Ensembl — https:\/\/www.ensembl.org\/Multi\/Search\/Results?q=GALC - Human Protein Atlas search — HPA — https:\/\/www.proteinatlas.org\/search\/GALC - PubMed (review) — NLM — https:\/\/pubmed.ncbi.nlm.nih.gov\/?term=GALC+review --\u003e","brand":"NSJ Bioreagents","offers":[{"title":"Adding 0.2 ml of distilled water will yield a concentration of 500 ug\/ml \/ 100 ug","offer_id":53047311204717,"sku":"FY13064","price":449.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0949\/7424\/7277\/files\/get_image_f3efe8a9-56ab-491a-830e-72b39a8d29b2.jpg?v=1782237082","url":"https:\/\/www.ebiohippo.com\/products\/galc-antibody-galactocerebrosidase-bha17135966","provider":"BioHippo","version":"1.0","type":"link"}