Metabolism Antibodies 1

Anti-AGL Antibody (CAB10051)

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SKU:
CAB10051
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Metabolism

Description

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Antibody Name:Anti-AGL Antibody
Antibody SKU:CAB10051
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1233-1532 of human AGL (NP_000633.2).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse
Positive Samples:293T, SW620, Mouse heart, Mouse skeletal muscle
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1233-1532 of human AGL (NP_000633.2).
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:EGFN ITAG VDEE TGFV YGGN RFNC GTWM DKMG ESDR ARNR GIPA TPRD GSAV EIVG LSKS AVRW LLEL SKKN IFPY HEVT VKRH GKAI KVSY DEWN RKIQ DNFE KLFH VSED PSDL NEKH PNLV HKRG IYKD SYGA SSPW CDYQ LRPN FTIA MVVA PELF TTEK AWKA LEIA EKKL LGPL GMKT LDPD DMVY CGIY DNAL DNDN YNLA KGFN YHQG PEWL WPIG YFLR AKLY FSRL MGPE TTAK TIVL VKNV LSRH YVHL ERSP WKGL PELT NENA QYCP FSCE TQAW SIAT ILET LYDL
Gene ID:178
Uniprot:P35573
Cellular Location:Cytoplasm
Calculated MW:172kDa/174kDa
Observed MW:174kDa
Synonyms:AGL, GDE
Background:This gene encodes the glycogen debrancher enzyme which is involved in glycogen degradation. This enzyme has two independent catalytic activities which occur at different sites on the protein: a 4-alpha-glucotransferase activity and a amylo-1, 6-glucosidase activity. Mutations in this gene are associated with glycogen storage disease although a wide range of enzymatic and clinical variability occurs which may be due to tissue-specific alternative splicing. Alternatively spliced transcripts encoding different isoforms have been described.
UniProt Protein Function:GDE: Multifunctional enzyme acting as 1,4-alpha-D-glucan:1,4- alpha-D-glucan 4-alpha-D-glycosyltransferase and amylo-1,6- glucosidase in glycogen degradation. Defects in AGL are the cause of glycogen storage disease type 3 (GSD3); also known as Forbes disease. GSD3 is a metabolic disorder associated with an accumulation of abnormal glycogen with short outer chains. Three GSD3 types are recognized: GSD type 3A patients lack glycogen debrancher enzyme activity in both liver and muscle, while GSD type 3B patients are enzyme- deficient in liver only. In rare cases, selective loss of only 1 of the 2 debranching activities, glucosidase or transferase, results in GSD type 3C or type 3D, respectively. GSD3 is clinically characterized by hepatomegaly, hypoglycemia, short stature, and variable myopathy. Belongs to the glycogen debranching enzyme family. 3 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Carbohydrate Metabolism - starch and sucrose; EC 3.2.1.33; EC 2.4.1.25; Ubiquitin conjugating system; Transferase; Hydrolase

Chromosomal Location of Human Ortholog: 1p21

Cellular Component: cytoplasm; cytosol; isoamylase complex; nucleus

Molecular Function:4-alpha-glucanotransferase activity; amylo-alpha-1,6-glucosidase activity; glycogen debranching enzyme activity; protein binding

Biological Process: glycogen catabolic process

Disease: Glycogen Storage Disease Iii

NCBI Summary:This gene encodes the glycogen debrancher enzyme which is involved in glycogen degradation. This enzyme has two independent catalytic activities which occur at different sites on the protein: a 4-alpha-glucotransferase activity and a amylo-1,6-glucosidase activity. Mutations in this gene are associated with glycogen storage disease although a wide range of enzymatic and clinical variability occurs which may be due to tissue-specific alternative splicing. Alternatively spliced transcripts encoding different isoforms have been described. [provided by RefSeq, Jul 2008]
UniProt Code:P35573
NCBI GenInfo Identifier:116242491
NCBI Gene ID:178
NCBI Accession:P35573.3
UniProt Secondary Accession:P35573,P78354, P78544, Q59H92, Q6AZ90, Q9UF08, A6NCX7 A6NEK2, D3DT51,
UniProt Related Accession:P35573
Molecular Weight:172,696 Da
NCBI Full Name:Glycogen debranching enzyme
NCBI Synonym Full Names:amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase
NCBI Official Symbol:AGL  
NCBI Official Synonym Symbols:GDE  
NCBI Protein Information:glycogen debranching enzyme
UniProt Protein Name:Glycogen debranching enzyme
UniProt Synonym Protein Names:Glycogen debrancherIncluding the following 2 domains:4-alpha-glucanotransferase (EC:2.4.1.25)Alternative name(s):Oligo-1,4-1,4-glucantransferase
UniProt Gene Name:AGL  
UniProt Entry Name:GDE_HUMAN
Anti-AGL Antibody (CAB10051)Western blot analysis of extracts of various cell lines, using AGL antibody (CAB10051) 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.
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