Anti-GBE1 Antibody (CAB6599)
- SKU:
- CAB6599
- Product type:
- Antibody
- Reactivity:
- Human
- Mouse
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Frequently bought together:
Description
| Antibody Name: | Anti-GBE1 Antibody |
| Antibody SKU: | CAB6599 |
| 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 1-300 of human GBE1 (NP_000149.3). |
| Application: | WB |
| Recommended Dilution: | WB 1:500 - 1:2000 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | HepG2, NCI-H460, Mouse heart, Mouse liver, Mouse kidney, Rat liver, Rat heart |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human GBE1 (NP_000149.3). |
| 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: | MAAP MTPA ARPE DYEA ALNA ALAD VPEL ARLL EIDP YLKP YAVD FQRR YKQF SQIL KNIG ENEG GIDK FSRG YESF GVHR CADG GLYC KEWA PGAE GVFL TGDF NGWN PFSY PYKK LDYG KWEL YIPP KQNK SVLV PHGS KLKV VITS KSGE ILYR ISPW AKYV VREG DNVN YDWI HWDP EHSY EFKH SRPK KPRS LRIY ESHV GISS HEGK VASY KHFT CNVL PRIK GLGY NCIQ LMAI MEHA YYAS FGYQ ITSF FAAS SRYG TPEE LQEL VDTA HSMG IIVL LDVV HSHA SKNS ADGL |
| Gene ID: | 2632 |
| Uniprot: | Q04446 |
| Cellular Location: | |
| Calculated MW: | 80kDa |
| Observed MW: | 80kDa |
| Synonyms: | GBE1, APBD, GBE, GSD4, 1 |
| Background: | The protein encoded by this gene is a glycogen branching enzyme that catalyzes the transfer of alpha-1, 4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1, 6 position on the same or a neighboring glycogen chain. Branching of the chains is essential to increase the solubility of the glycogen molecule and, consequently, in reducing the osmotic pressure within cells. Highest level of this enzyme are found in liver and muscle. Mutations in this gene are associated with glycogen storage disease IV (also known as Andersen's disease). |
| UniProt Protein Function: | GBE1: a monomeric enzyme that functions in glycogen symthesis by catalyzing the formation of alpha 1,6- glucosidic linkages.Required for sufficient glycogen accumulation. The alpha 1-6 branches of glycogen play an important role in increasing the solubility of the molecule and, consequently, in reducing the osmotic pressure within cells. It is most highly expressed in liver and muscle. Deficiency can result in glycogen storage disease IV (Andersen's disease). |
| UniProt Protein Details: | Protein type:EC 2.4.1.18; Carbohydrate Metabolism - starch and sucrose; Transferase Chromosomal Location of Human Ortholog: 3p12.3 Cellular Component: cytosol Molecular Function:cation binding; 1,4-alpha-glucan branching enzyme activity; hydrolase activity, hydrolyzing O-glycosyl compounds Biological Process: glycogen metabolic process; generation of precursor metabolites and energy; glycogen biosynthetic process; carbohydrate metabolic process; glucose metabolic process; pathogenesis Disease: Polyglucosan Body Disease, Adult Form; Glycogen Storage Disease Iv |
| NCBI Summary: | The protein encoded by this gene is a glycogen branching enzyme that catalyzes the transfer of alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position on the same or a neighboring glycogen chain. Branching of the chains is essential to increase the solubility of the glycogen molecule and, consequently, in reducing the osmotic pressure within cells. Highest level of this enzyme are found in liver and muscle. Mutations in this gene are associated with glycogen storage disease IV (also known as Andersen's disease). [provided by RefSeq, Jul 2008] |
| UniProt Code: | Q04446 |
| NCBI GenInfo Identifier: | 357529509 |
| NCBI Gene ID: | 2632 |
| NCBI Accession: | Q04446.3 |
| UniProt Secondary Accession: | Q04446,Q96EN0, B3KWV3, |
| UniProt Related Accession: | Q04446 |
| Molecular Weight: | 702 |
| NCBI Full Name: | 1,4-alpha-glucan-branching enzyme |
| NCBI Synonym Full Names: | glucan (1,4-alpha-), branching enzyme 1 |
| NCBI Official Symbol: | GBE1 |
| NCBI Official Synonym Symbols: | GBE; APBD; GSD4 |
| NCBI Protein Information: | 1,4-alpha-glucan-branching enzyme; brancher enzyme; glycogen branching enzyme; glycogen-branching enzyme; amylo-(1,4 to 1,6) transglucosidase; amylo-(1,4 to 1,6) transglycosylase |
| UniProt Protein Name: | 1,4-alpha-glucan-branching enzyme |
| UniProt Synonym Protein Names: | Brancher enzyme; Glycogen-branching enzyme |
| Protein Family: | 1,4-alpha-glucan branching enzyme |
| UniProt Gene Name: | GBE1 |
| UniProt Entry Name: | GLGB_HUMAN |
 | Western blot analysis of extracts of various cell lines, using GBE1 antibody (CAB6599) 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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