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Mouse Anti-FUCA1 Recombinant Antibody (CBYJT-1284) (CBMAB-T0299-YJ)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBYJT-1284
Antibody Isotype
IgG1
Application
WB

Basic Information

Immunogen
Mouse myeloma cell line NS0-derived recombinant human Tissue alpha-L-Fucosidase/FUCA1, Gln32-Lys466, Accession # P04066
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Buffer
PBS, Trehalose
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
aa 32-466

Target

Full Name
Alpha-L-Fucosidase 1
Introduction
FUCA1 is a lysosomal enzyme involved in the degradation of fucose-containing glycoproteins and glycolipids. Mutations in this gene are associated with fucosidosis (FUCA1D), which is an autosomal recessive lysosomal storage disease.
Entrez Gene ID
2517
UniProt ID
P04066
Alternative Names
Alpha-L-Fucosidase 1; Alpha-L-Fucoside Fucohydrolase 1; Fucosidase, Alpha-L- 1, Tissue; Alpha-L-Fucosidase I; Tissue Fucosidase; A-L-Fucosidase 1
Function
Alpha-L-fucosidase is responsible for hydrolyzing the alpha-1,6-linked fucose joined to the reducing-end N-acetylglucosamine of the carbohydrate moieties of glycoproteins.
Biological Process
Fucose metabolic process Source: UniProtKB
Glycolipid catabolic process Source: BHF-UCL
Glycosaminoglycan catabolic process Source: UniProtKB
Glycoside catabolic process Source: UniProtKB
Cellular Location
Lysosome
Involvement in disease
Fucosidosis (FUCA1D):
An autosomal recessive lysosomal storage disease characterized by accumulation of fucose-containing glycolipids and glycoproteins in various tissues. Clinical signs include facial dysmorphism, dysostosis multiplex, moderate hepatomegaly, severe intellectual deficit, deafness, and according to age, angiokeratomas.
More Infomation

Xiao, Y., Jiang, X., Yin, K., Miao, T., Lu, H., Wang, W., ... & Zhang, P. (2023). USP35 promotes cell proliferation and chemotherapeutic resistance through stabilizing FUCA1 in colorectal cancer. Oncogenesis, 12(1), 12.

Armstrong, Z., Meek, R. W., Wu, L., Blaza, J. N., & Davies, G. J. (2022). Cryo-EM structures of human fucosidase FucA1 reveal insight into substrate recognition and catalysis. Structure, 30(10), 1443-1451.

Barelier, S., & Sulzenbacher, G. (2022). The long-awaited structure of human fucosidase FucA1 opens novel avenues for the treatment of fucosidosis. Structure, 30(10), 1369-1371.

Chkioua, L., Amri, Y., Saheli, C., Fenni, F., Boudabous, H., Ben Turkia, H., ... & Laradi, S. (2021). Fucosidosis in Tunisian patients: mutational analysis and homology-based modeling of FUCA1 enzyme. BMC medical genomics, 14, 1-11.

Zhang, X., Zhao, S., Liu, H., Wang, X., Wang, X., Du, N., ... & Duan, H. (2021). Identification of a novel homozygous loss-of-function mutation in FUCA1 gene causing severe fucosidosis: A case report. Journal of International Medical Research, 49(4), 03000605211005975.

Domin, A., Zabek, T., Kwiatkowska, A., Szmatola, T., Deregowska, A., Lewinska, A., ... & Wnuk, M. (2021). The Identification of a Novel Fucosidosis-Associated FUCA1 Mutation: A Case of a 5-Year-Old Polish Girl with Two Additional Rare Chromosomal Aberrations and Affected DNA Methylation Patterns. Genes, 12(1), 74.

Wali, G., Wali, G. M., Sue, C. M., & Kumar, K. R. (2019). A novel homozygous mutation in the FUCA1 gene highlighting fucosidosis as a cause of dystonia: Case report and literature review. Neuropediatrics, 50(04), 248-252.

Bonin, S., Parascandolo, A., Aversa, C., Barbazza, R., Tsuchida, N., Castellone, M. D., ... & Vecchio, G. (2018). Reduced expression of α-L-Fucosidase-1 (FUCA-1) predicts recurrence and shorter cancer specific survival in luminal B LN+ breast cancer patients. Oncotarget, 9(20), 15228.

Valero‐Rubio, D., Jiménez, K. M., Fonseca, D. J., Payán‐Gómez, C., & Laissue, P. (2018). Transcriptomic analysis of FUCA 1 knock‐down in keratinocytes reveals new insights into the pathogenesis of fucosidosis skin lesions. Experimental dermatology, 27(6), 663-667.

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For research use only. Not intended for any clinical use.

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