Cell Biology Antibodies 4

Anti-AGA Antibody (CAB13942)

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SKU:
CAB13942
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cell Biology

Description

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Antibody Name:Anti-AGA Antibody
Antibody SKU:CAB13942
Antibody Size:20uL, 50uL, 100uL
Application:WB IF
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 24-346 of human AGA (NP_000018.2).
Application:WB IF
Recommended Dilution:WB 1:500 - 1:1000 IF 1:50 - 1:100
Reactivity:Human, Mouse
Positive Samples:SW620, THP-1, HepG2, Mouse liver, Mouse pancreas
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 24-346 of human AGA (NP_000018.2).
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:SSPL PLVV NTWP FKNA TEAA WRAL ASGG SALD AVES GCAM CERE QCDG SVGF GGSP DELG ETTL DAMI MDGT TMDV GAVG DLRR IKNA IGVA RKVL EHTT HTLL VGES ATTF AQSM GFIN EDLS TTAS QALH SDWL ARNC QPNY WRNV IPDP SKYC GPYK PPGI LKQD IPIH KETE DDRG HDTI GMVV IHKT GHIA AGTS TNGI KFKI HGRV GDSP IPGA GAYA DDTA GAAA ATGN GDIL MRFL PSYQ AVEY MRRG EDPT IACQ KVIS RIQK HFPE FFGA VICA NVTG SYGA ACNK LSTF TQFS FMVY NSEK NQPT EEKV DCI
Gene ID:175
Uniprot:P20933
Cellular Location:Lysosome
Calculated MW:37kDa
Observed MW:18kDa
Synonyms:AGA, AGU, ASRG, GA
Background:This gene encodes a member of the N-terminal nucleophile (Ntn) hydrolase family of proteins. The encoded preproprotein is proteolytically processed to generate alpha and beta chains that comprise the mature enzyme. This enzyme is involved in the catabolism of N-linked oligosaccharides of glycoproteins. It cleaves asparagine from N-acetylglucosamines as one of the final steps in the lysosomal breakdown of glycoproteins. Mutations in this gene are associated with the lysosomal storage disease aspartylglycosaminuria that results in progressive neurodegeneration. Alternative splicing results in multiple transcript variants, at least one of which encodes an isoform that is subject to proteolytic processing.
UniProt Protein Function:AGA: a catabolic enzyme, aspartylglucosaminidase is involved in the catabolism of N-linked oligosaccharides of glycoproteins. It cleaves asparagine from N-acetylglucosamines as one of the final steps in the lysosomal breakdown of glycoproteins. The lysosomal storage disease aspartylglycosaminuria is caused by a deficiency in the AGA enzyme.
UniProt Protein Details:

Protein type:Glycan Metabolism - other glycan degradation; EC 3.5.1.26; Hydrolase

Chromosomal Location of Human Ortholog: 4q34.3

Cellular Component: endoplasmic reticulum; lysosome

Molecular Function:peptidase activity; N4-(beta-N-acetylglucosaminyl)-L-asparaginase activity; protein self-association

Biological Process: protein deglycosylation; protein maturation; proteolysis

Disease: Aspartylglucosaminuria

NCBI Summary:Aspartylglucosaminidase is involved in the catabolism of N-linked oligosaccharides of glycoproteins. It cleaves asparagine from N-acetylglucosamines as one of the final steps in the lysosomal breakdown of glycoproteins. The lysosomal storage disease aspartylglycosaminuria is caused by a deficiency in the AGA enzyme. Alternatively spliced transcript variants have been identified. [provided by RefSeq, Jan 2010]
UniProt Code:P20933
NCBI GenInfo Identifier:288558804
NCBI Gene ID:175
NCBI Accession:P20933.2
UniProt Secondary Accession:P20933,Q4W5Q2, Q6FHN6, Q9UCK6, Q9UCK7, Q9UCK8, B2R7H2 D3DP47,
UniProt Related Accession:P20933
Molecular Weight:346
NCBI Full Name:N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase
NCBI Synonym Full Names:aspartylglucosaminidase
NCBI Official Symbol:AGA  
NCBI Official Synonym Symbols:GA; AGU; ASRG  
NCBI Protein Information:N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase; N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase; glycosylasparaginase; aspartylglucosylamine deaspartylase; N4-(N-acetyl-beta-glucosaminyl)-L-asparagine amidase
UniProt Protein Name:N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase
UniProt Synonym Protein Names:Aspartylglucosaminidase; Glycosylasparaginase; N4-(N-acetyl-beta-glucosaminyl)-L-asparagine amidaseCleaved into the following 2 chains:Glycosylasparaginase alpha chain; Glycosylasparaginase beta chain
Protein Family:Agaricus bisporus lectin
UniProt Gene Name:AGA  
UniProt Entry Name:ASPG_HUMAN
Anti-AGA Antibody (CAB13942)Western blot analysis of extracts of various cell lines, using AGA antibody (CAB13942) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.
Anti-AGA Antibody (CAB13942)Immunofluorescence analysis of HeLa cells using AGA antibody (CAB13942). Blue: DAPI for nuclear staining.
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