EAW95098.1
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
53,689 Da
NCBI Official Full Name
fucosidase, alpha-L- 1, tissue
NCBI Official Synonym Full Names
fucosidase, alpha-L- 1, tissue
NCBI Official Synonym Symbols
NCBI Protein Information
tissue alpha-L-fucosidase; alpha-L-fucosidase 1; alpha-L-fucosidase I; alpha-L-fucoside fucohydrolase 1
UniProt Protein Name
Tissue alpha-L-fucosidase
UniProt Synonym Protein Names
Alpha-L-fucosidase I; Alpha-L-fucoside fucohydrolase 1; Alpha-L-fucosidase 1
UniProt Synonym Gene Names
UniProt Entry Name
FUCO_HUMAN
NCBI Summary for FUCa1
The protein encoded by this gene 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. A pseudogene of this locus is present on chr 2.[provided by RefSeq, Oct 2009]
UniProt Comments for FUCa1
FUCA1: 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. Defects in FUCA1 are the cause of fucosidosis (FUCA1D). FUCA1D is 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. Belongs to the glycosyl hydrolase 29 family.
Protein type: Hydrolase; EC 3.2.1.51; Glycan Metabolism - other glycan degradation
Chromosomal Location of Human Ortholog: 1p34
Cellular Component: lysosome; cytoplasm
Molecular Function: alpha-L-fucosidase activity; fucose binding
Biological Process: fucose metabolic process; glycosaminoglycan catabolic process; glycoside catabolic process
Disease: Fucosidosis
Research Articles on FUCa1
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Products associated with FUCa1 elisa kit
Pathways associated with FUCa1 elisa kit
Diseases associated with FUCa1 elisa kit
Organs/Tissues associated with FUCa1 elisa kit
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