Human FUCA1 Recombinant Protein (RPPB1650)
- SKU:
- RPPB1650
- Product Type:
- Recombinant Protein
- Species:
- Human
- Uniprot:
- P04066
- Research Area:
- Enzymes
Description
| 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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