GALC Antibody (2D1): sc-293200

GALC Antibody (2D1) is a mouse monoclonal IgG2a kappa light chain antibody that detects GALC protein of human origin by western blotting (WB), immunoprecipitation (IP), and enzyme-linked immunosorbent assay (ELISA). Anti-GALC antibody (2D1) is available as a non-conjugated isotype antibody. GALC protein, also known as galactosylceramidase, plays a crucial role as a lysosomal enzyme responsible for hydrolyzing galactose ester bonds in various galactolipids, including galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. These galactolipids are essential components of the myelin sheath, which insulates nerve fibers and facilitates efficient signal transmission in the nervous system. Proper functioning of GALC is vital for maintaining myelin integrity and overall neurological health. Mutations in the GALC gene that impair enzymatic activity are linked to Krabbe disease, a severe autosomal recessive disorder that leads to progressive neurological degeneration, affecting approximately 1 in 150,000 infants. The "twitcher" mouse model, characterized by a specific mutation in the GALC gene, serves as a valuable tool for studying the disease and potential therapeutic interventions. Alternate splice variants of the GALC gene have been identified, which may encode different isoforms with distinct functional properties, further emphasizing the complexity and importance of GALC in cellular processes.

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GALC Antibody (2D1) References:

1. Suppression of galactosylceramidase (GALC) expression in the twitcher mouse model of globoid cell leukodystrophy (GLD) is caused by nonsense-mediated mRNA decay (NMD).  |  Lee, WC., et al. 2006. Neurobiol Dis. 23: 273-80. PMID: 16759875
2. Altered Trafficking and Processing of GALC Mutants Correlates with Globoid Cell Leukodystrophy Severity.  |  Shin, D., et al. 2016. J Neurosci. 36: 1858-70. PMID: 26865610
3. GALC Triggers Tumorigenicity of Colorectal Cancer via Senescent Fibroblasts.  |  Yang, M., et al. 2020. Front Oncol. 10: 380. PMID: 32318333
4. Neuron-specific ablation of the Krabbe disease gene galactosylceramidase in mice results in neurodegeneration.  |  Kreher, C., et al. 2022. PLoS Biol. 20: e3001661. PMID: 35789331
5. Galactosylceramidase deficiency and pathological abnormalities in cerebral white matter of Krabbe disease.  |  Iacono, D., et al. 2022. Neurobiol Dis. 174: 105862. PMID: 36113749
6. GALC variants affect galactosylceramidase enzymatic activity and risk of Parkinson's disease.  |  Senkevich, K., et al. 2023. Brain. 146: 1859-1872. PMID: 36370000
7. The Pro-Oncogenic Sphingolipid-Metabolizing Enzyme β-Galactosylceramidase Modulates the Proteomic Landscape in BRAF(V600E)-Mutated Human Melanoma Cells.  |  Capoferri, D., et al. 2023. Int J Mol Sci. 24: PMID: 37445731
8. Deficiency of galactosyl-ceramidase in adult oligodendrocytes worsens disease severity during chronic experimental allergic encephalomyelitis.  |  Saldivia, N., et al. 2024. Mol Ther. 32: 3163-3176. PMID: 38937968
9. Lysosomal TMEM106B interacts with galactosylceramidase to regulate myelin lipid metabolism.  |  Takahashi, H., et al. 2024. Commun Biol. 7: 1088. PMID: 39237682
10. Expression study of Krabbe Disease GALC missense variants - Insights from quantification profiles of residual enzyme activity, secretion and psychosine levels.  |  Peng, H., et al. 2024. bioRxiv.. PMID: 39464077

The preferred GALC antibody is GALC Antibody (G-6): sc-518055.