Cell Biology Antibodies 10

Anti-FUCA1 Antibody (CAB6291)

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SKU:
CAB6291
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cell Biology

Description

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Antibody Name:Anti-FUCA1 Antibody
Antibody SKU:CAB6291
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 20-260 of human FUCA1 (NP_000138.2).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse, Rat
Positive Samples:
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 20-260 of human FUCA1 (NP_000138.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:LFLG AAES VRRA QPPR RYTP DWPS LDSR PLPA WFDE AKFG VFIH WGVF SVPA WGSE WFWW HWQG EGRP QYQR FMRD NYPP GFSY ADFG PQFT ARFF HPEE WADL FQAA GAKY VVLT TKHH EGFT NWPS PVSW NWNS KDVG PHRD LVGE LGTA LRKR NIRY GLYH SLLE WFHP LYLL DKKN GFKT QHFV SAKT MPEL YDLV NSYK PDLI WSDG EWEC PDTY WNST NFLS WLYN DSPV KDEV V
Gene ID:2517
Uniprot:P04066
Cellular Location:Lysosome
Calculated MW:53kDa
Observed MW:Refer to figures
Synonyms:FUCA1, FUCA
Background: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.
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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