Anti-AGL Antibody (CAB10051)
- SKU:
- CAB10051
- Product type:
- Antibody
- Reactivity:
- Human
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Frequently bought together:
Description
| 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 |
 | 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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