Enzymes Recombinant Proteins

Human FUCA1 Recombinant Protein (RPPB1650)

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RPPB1650

Description

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Product Name:Human FUCA1 Recombinant Protein
Product Code:RPPB1650
Size:5µg
Species:Human
Target:FUCA1
Synonyms:Fucosidase, Alpha-L- 1, Tissue, Alpha-L-FucosideFucohydrolase 1, Alpha-L-Fucosidase 1, Alpha-L-Fucosidase I, EC 3.2.1.51, TissueAlpha-L-Fucosidase, EC 3.2.1, FUCA, Tissue alpha-L-fucosidase.
Source:Sf9 Insect cells
Physical Appearance:Sterile Filtered colorless solution.
Formulation:FUCA1 protein solution (0.5mg/ml) contains PhosphateBuffered Saline (pH 7.4) and 10% glycerol.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 90% as determined by SDS-PAGE.
Amino Acid Sequence:VRRAQPPRRY TPDWPSLDSR PLPAWFDEAK FGVFIHWGVF SVPAWGSEWFWWHWQGEGRP QYQRFMRDNY PPGFSYADFG PQFTARFFHP EEWADLFQAA GAKYVVLTTK HHEGFTNWPSPVSWNWNSKD VGPHRDLVGE LGTALRKRNI RYGLYHSLLE WFHPLYLLDK KNGFKTQHFV SAKTMPELYDLVNSYKPDLI WSDGEWECPD TYWNSTNFLS WLYNDSPVKD EVVVNDRWGQ NCSCHHGGYY NCEDKFKPQSLPDHKWEMCT SIDKFSWGYR RDMALSDVTE ESEIISELVQ TVSLGGNYLL NIGPTKDGLI VPIFQERLLAVGKWLSINGE AIYASKPWRV QWEKNTTSVW YTSKGSAVYA IFLHWPENGV LNLESPITTS TTKITMLGIQGDLKWSTDPD KGLFISLPQL PPSAVPAEFA WTIKLTGVKH HHHHH

Fucosidase Alpha-L- 1 Plasma, alsoknown as FUCA1 is a member of the glycosyl hydrolase 29 family which isresponsible for hydrolyzing the alpha-1,6-linked fucose joined to the reducing-end N-acetylglucosamine of thecarbohydrate moieties of glycoproteins. Fucosidosis is an autosomal recessivelysosomal storage disease caused by the absence of alpha-L-fucosidase activity.

FUCA1 produced in Sf9 Baculovirus cells is a single,glycosylated polypeptide chain containing 445 amino acids (28-466a.a.) andhaving a molecular mass of 51.7kDa.�(Molecular size on SDS-PAGE will appear atapproximately 50-70kDa).�FUCA1 is expressed with a 6 amino acid His tag atC-Terminus and purified by proprietary chromatographic techniques.

UniProt Protein Function: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.
UniProt Protein Details:

Protein type:Glycan Metabolism - other glycan degradation; EC 3.2.1.51; Hydrolase

Chromosomal Location of Human Ortholog: 1p34

Cellular Component: cytoplasm; lysosomal lumen

Molecular Function:alpha-L-fucosidase activity

Biological Process: fucose metabolic process; glycoside catabolic process

Disease: Fucosidosis

NCBI Summary: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 Code:P04066
NCBI GenInfo Identifier:156631012
NCBI Gene ID:2517
NCBI Accession:P04066.4
UniProt Secondary Accession:P04066,Q14334, Q14335, Q3LID0, Q8NAC2, B2RBG3,
UniProt Related Accession:P04066
Molecular Weight:53,689 Da
NCBI Full Name:Tissue alpha-L-fucosidase
NCBI Synonym Full Names:fucosidase, alpha-L- 1, tissue
NCBI Official Symbol:FUCA1��
NCBI Official Synonym Symbols:FUCA��
NCBI Protein Information:tissue alpha-L-fucosidase
UniProt Protein Name:Tissue alpha-L-fucosidase
UniProt Synonym Protein Names:Alpha-L-fucosidase I; Alpha-L-fucoside fucohydrolase 1; Alpha-L-fucosidase 1
Protein Family:Tissue alpha-L-fucosidase
UniProt Gene Name:FUCA1��
UniProt Entry Name:FUCO_HUMAN
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Additional Information

Product Type:
Recombinant Protein
Species:
Human
Uniprot:
P04066
Research Area:
Enzymes
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