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Glucan (1,4-alpha-), Branching Enzyme 1 Proteins (GBE1)

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 Antibodies (70) and Glucan (1,4-alpha-), Branching Enzyme 1 Kits (17) and many more products for this protein.

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

Showing 4 out of 5 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
HOST_Escherichia coli (E. coli) Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 39 to 44 Days
$9,248.02
Details
HOST_Escherichia coli (E. coli) Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 39 to 44 Days
$9,248.02
Details
HOST_Wheat germ Human GST tag 10 μg Log in to see 9 Days
$405.71
Details
HOST_Human Human Un-conjugated   20 μg Log in to see 9 to 11 Days
$785.40
Details

GBE1 Proteins by Origin and Source

Origin Expressed in Conjugate
Human , ,
,
Mouse (Murine)

More Proteins for Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) 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 Proteins) 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 Proteins) 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 Proteins) 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 Proteins) storage disease type IV

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

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

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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