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anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) Antibodies

The protein encoded by GBE1 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. Additionally we are shipping Glucan (1,4-alpha-), Branching Enzyme 1 Kits (17) and Glucan (1,4-alpha-), Branching Enzyme 1 Proteins (5) and many more products for this protein.

list all antibodies Gene Name GeneID UniProt
GBE1 2632 Q04446
GBE1 74185 Q9D6Y9
GBE1 288333  
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Top anti-Glucan (1,4-alpha-), Branching Enzyme 1 Antibodies at antibodies-online.com

Showing 10 out of 74 products:

Catalog No. Reactivity Host Conjugate Application Images Quantity Supplier Delivery Price Details
Human Rabbit Un-conjugated EIA, IF, WB Confocal immunofluorescent analysis of GBE1 Antibody (Center) Cat.-No AP51790PU-N with HepG2 cell followed by Alexa Fluor? 488-conjugated goat anti-rabbit lgG (green). Actin filaments have been labeled with Alexa Fluor 555 phalloidin (red). DAPI was used to stain the cell nuclear (blue). Western blot analysis of GBE1 Antibody (Center) in HL-60 cell line lysates (35ug/lane). 0.4 mL Log in to see 6 to 8 Days
$390.50
Details
Human Mouse Un-conjugated IP, IHC, WB Immunohistochemical staining of paraffin-embedded Kidney tissue using anti-GBE1 mouse monoclonal antibody. (ABIN2453063, Dilution 1:50) Immunohistochemical staining of paraffin-embedded liver tissue using anti-GBE1 mouse monoclonal antibody. (ABIN2453063, Dilution 1:50) 100 μL Log in to see 2 to 3 Days
$423.50
Details
Human Mouse Un-conjugated FACS, IF, IP, WB Anti-GBE1 mouse monoclonal antibody (ABIN2453066) immunofluorescent staining of COS7 cells transiently transfected by pCMV6-ENTRY GBE1 (RC204152). HEK293T cells were transfected with the pCMV6-ENTRY control (Left lane) or pCMV6-ENTRY GBE1 (Right lane) cDNA for 48 hrs and lysed. Equivalent amounts of cell lysates (5 µg per lane) were separated by SDS-PAGE and immunoblotted with anti-GBE1. 100 μL Log in to see 2 to 3 Days
$423.50
Details
Human Mouse Un-conjugated FACS, IF, IP, IHC, WB Immunohistochemical staining of paraffin-embedded colon tissue using anti-GBE1mouse monoclonal antibody. (ABIN2453067, Dilution 1:50) Immunohistochemical staining of paraffin-embedded Ovary tissue using anti-GBE1mouse monoclonal antibody. (ABIN2453067, Dilution 1:50) 100 μL Log in to see 2 to 3 Days
$482.90
Details
Human Rabbit Un-conjugated WB 100 μL Log in to see 2 to 3 Days
$289.00
Details
Human Mouse Un-conjugated ELISA, WB Western Blot detection against Immunogen (36.89 KDa) . 50 μL Log in to see 9 Days
$298.57
Details
Human Rabbit Un-conjugated IF, WB Western blot analysis of GBE1 Antibody (Center) (ABIN653459) in HL-60 cell line lysates (35 µg/lane). GBE1 (arrow) was detected using the purified polyclonal antibody. Western blot analysis of GBE1 Antibody (Center) (ABIN653459) in mouse liver tissue lysates (35 µg/lane).GBE1 (arrow) was detected using the purified polyclonal antibody. 400 μL Log in to see 10 to 11 Days
$324.50
Details
Human Mouse Un-conjugated IHC (p), IP, WB 100 μL Log in to see 8 to 10 Days
$493.17
Details
Human Mouse Un-conjugated FACS, IHC (p), IP, WB 100 μL Log in to see 8 to 10 Days
$493.17
Details
Human Mouse Un-conjugated FACS, IP, IHC, WB Immunohistochemical staining of paraffin-embedded liver tissue using anti-GBE1 mouse monoclonal antibody. (ABIN2453064, Dilution 1:50) Immunohistochemical staining of paraffin-embedded Ovary tissue using anti-GBE1 mouse monoclonal antibody. (ABIN2453064, Dilution 1:50) 100 μL Log in to see 2 to 3 Days
$423.50
Details

GBE1 Antibodies by Reactivity, Application, Clonality and Conjugate

Attributes Applications Host Clonality Conjugate
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Top referenced anti-Glucan (1,4-alpha-), Branching Enzyme 1 Antibodies

  1. Human Polyclonal GBE1 Primary Antibody for EIA, IF - ABIN952469 : Konstantinidou, Anninos, Dertinger, Nonni, Petersen, Karadimas, Havaki, Marinos, Akman, DiMauro, Patsouris: Placental involvement in glycogen storage disease type IV. in Placenta 2008 (PubMed)
    Show all 3 references for ABIN952469

  2. Human Polyclonal GBE1 Primary Antibody for ELISA, WB - ABIN561010 : Kakhlon, Glickstein, Feinstein, Liu, Baba, Terashima, Akman, Dimauro, Lossos: Polyglucosan neurotoxicity caused by glycogen branching enzyme deficiency can be reversed by inhibition of glycogen synthase. in Journal of neurochemistry 2013 (PubMed)
    Show all 2 references for ABIN561010

  3. Human Monoclonal GBE1 Primary Antibody for FACS, IF - ABIN2453067 : Akman, Sheiko, Tay, Finegold, Dimauro, Craigen: Generation of a novel mouse model that recapitulates early and adult onset glycogenosis type IV. in Human molecular genetics 2011 (PubMed)

  4. Human Monoclonal GBE1 Primary Antibody for FACS, IF - ABIN2453066 : Colombo, Pagliarani, Testolin, Salsano, Napoli, Bordoni, Salani, DAdda, Morandi, Farina, Magri, Riva, Prelle, Sciacco, Comi, Moggio: Adult polyglucosan body disease: clinical and histological heterogeneity of a large Italian family. in Neuromuscular disorders : NMD 2015 (PubMed)

More Antibodies against Glucan (1,4-alpha-), Branching Enzyme 1 Interaction Partners

Human Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) interaction partners

  1. The crystal structure of GBE1 in complex with oligosaccharides was determined, the structural and molecular bases of Adult Polyglucosan Body Disease-linked missense mutations was investigated.

  2. The presence of polyglucosan bodies in intramuscular nerve twigs by itself and is not an indication of APBD mutation.

  3. GBE1 mutation is found in manifesting heterozygous patients with adult polyglucosan body disease

  4. Case Reports: novel missense/deletion mutations in GBE1 in glycogen (show GYS1 Antibodies) storage disease type IV.

  5. GBE1 mutations can cause an early adult-onset relapsing-remitting form of polyglucosan body disease distinct from adult polyglucosan body disease in several ways, including younger age at onset.

  6. Compound heterozygous mutations in GBE1 were identified as the cause of lethal multiple pterygium syndrome in a family.

  7. this is the first epidemiologic study of the mutation frequency of the adult polyglucosan body disease -associated GBE1 mutation c.1076A>C in a large Ashkenazi Jewish cohort.

  8. APBD with GBE deficiency is a clinically homogenous disorder that should be suspected in patients with adult onset leukodystrophy or spastic paraplegia with early onset of urinary symptoms and spinal atrophy.

  9. A review of the literature for glycogen (show GYS1 Antibodies) storage disease type IV patients with characterized molecular defects and deficient enzyme activity reveals most GBE1 mutations to be missense mutations clustering in the catalytic enzyme domain.

  10. Case Report: report an as yet undefined and different phenotype of glycogen (show GYS1 Antibodies) storage disease with diminished branching enzyme activity associated with multisystemic involvement.

Mouse (Murine) Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) interaction partners

  1. results reveal that early molecular events associated with Gbe1 deficiency contribute to abnormal cardiac development and fetal hydrops in the fetal form of glycogen (show GYS1 Antibodies) storage disease type IV

  2. GYS1 (show GYS1 Antibodies) regulation by HIF plays a central role in the hypoxic accumulation of glycogen (show GYS1 Antibodies), and hypoxia also upregulates the expression of UTP:glucose-1-phosphate urydylyltransferase (UGP2 (show UGP2 Antibodies)) and 1,4-alpha glucan branching enzyme (GBE1)

Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) Antigen Profile

Protein 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).

Gene names and symbols associated with GBE1

  • glycogen branching enzyme (glgB) antibody
  • glycogen branching enzyme (ECs4277) antibody
  • glycogen branching protein (SCO7332) antibody
  • glycogen branching enzyme; BE; 1,4-alpha-D-glucan:1,4-alpha-D-glucan 6-glucosyl-transferase (glgB) antibody
  • glycogen branching enzyme (Synpcc7942_1085) antibody
  • glycogen branching enzyme (AZC_4116) antibody
  • glycogen branching enzyme (Sros_8169) antibody
  • glycogen branching enzyme (Despr_0975) antibody
  • glucan (1,4-alpha-), branching enzyme 1 (GBE1) antibody
  • glucan (1,4-alpha-), branching enzyme 1 (Gbe1) antibody
  • 2310045H19Rik antibody
  • 2810426P10Rik antibody
  • APBD antibody
  • D16Ertd536e antibody
  • GBE antibody
  • glgBII antibody
  • GSD4 antibody

Protein level used designations for GBE1

glycogen branching enzyme , 1,4-alpha-glucan-branching enzyme , amylo-(1,4 to 1,6) transglucosidase , amylo-(1,4 to 1,6) transglycosylase , brancher enzyme , glycogen-branching enzyme , glucan (1,4-alpha-), branching enzyme 1 (glycogen branching enzyme, Andersen disease, glycogen storage disease type IV)

GENE ID SPECIES
881350 Pseudomonas aeruginosa PAO1
886893 Mycobacterium tuberculosis H37Rv
915869 Escherichia coli O157:H7 str. Sakai
950275 Haemophilus influenzae Rd KW20
952758 Synechocystis sp. PCC 6803
1102770 Streptomyces coelicolor A3(2)
3198206 Synechococcus elongatus PCC 6301
3431022 Haemophilus influenzae 86-028NP
3775035 Synechococcus elongatus PCC 7942
5586828 Escherichia coli E24377A
5688468 Azorhizobium caulinodans ORS 571
8671497 Streptosporangium roseum DSM 43021
10173693 Desulfobulbus propionicus DSM 2032
2632 Homo sapiens
74185 Mus musculus
493962 Felis catus
100034152 Equus caballus
288333 Rattus norvegicus
101120124 Ovis aries
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