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1,4-Alpha-Glucan Branching Enzyme 1 (GBE1)

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Glycogen Branching Enzyme; Andersen Disease; Glycogen Storage Disease Type IV; Brancher enzyme

1,4-Alpha-Glucan Branching Enzyme 1 (GBE1)
CCC J063
HGNC 4180
MGI 1921435
PubMed 23218673
RGD 1309968
UniProt Q04446
Wiki GBE1
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. 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. The human and yeast glycogen branching enzymes were found to have 67% identical amino acid sequence over a major portion of their length. The full length of the cDNA was approximately 3 kb. The coding sequence contained 2,106 bp encoding a 702-amino acid protein. The calculated molecular mass of the GBE1 protein, derived from its cDNA sequence, was 80,438 Da.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant 1,4-Alpha-Glucan Branching Enzyme 1 (GBE1) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to 1,4-Alpha-Glucan Branching Enzyme 1 (GBE1) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to 1,4-Alpha-Glucan Branching Enzyme 1 (GBE1) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for 1,4-Alpha-Glucan Branching Enzyme 1 (GBE1) CLIA Kit Customized Service Offer
n/a ELISA Kit for 1,4-Alpha-Glucan Branching Enzyme 1 (GBE1) ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant 1,4-Alpha-Glucan Branching Enzyme 1 (GBE1) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to 1,4-Alpha-Glucan Branching Enzyme 1 (GBE1) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to 1,4-Alpha-Glucan Branching Enzyme 1 (GBE1) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for 1,4-Alpha-Glucan Branching Enzyme 1 (GBE1) CLIA Kit Customized Service Offer
n/a ELISA Kit for 1,4-Alpha-Glucan Branching Enzyme 1 (GBE1) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant 1,4-Alpha-Glucan Branching Enzyme 1 (GBE1) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to 1,4-Alpha-Glucan Branching Enzyme 1 (GBE1) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to 1,4-Alpha-Glucan Branching Enzyme 1 (GBE1) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for 1,4-Alpha-Glucan Branching Enzyme 1 (GBE1) CLIA Kit Customized Service Offer
n/a ELISA Kit for 1,4-Alpha-Glucan Branching Enzyme 1 (GBE1) ELISA Kit Customized Service Offer
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  2. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
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  7. "Comparative large-scale characterisation of plant vs. mammal proteins reveals similar and idiosyncratic N-alpha acetylation features."Mol. Cell. Proteomics 11:M111.015131-M111.015131(2012) [PubMed] [Europe PMC] [Abstract]
  8. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  9. "Hepatic and neuromuscular forms of glycogen storage disease type IV caused by mutations in the same glycogen-branching enzyme gene."J. Clin. Invest. 97:941-948(1996) [PubMed] [Europe PMC] [Abstract]
  10. "A novel missense mutation in the glycogen branching enzyme gene in a child with myopathy and hepatopathy."Neuromuscul. Disord. 9:403-407(1999) [PubMed] [Europe PMC] [Abstract]
  11. "Novel missense mutations in the glycogen-branching enzyme gene in adult polyglucosan body disease."Ann. Neurol. 47:536-540(2000) [PubMed] [Europe PMC] [Abstract]
  12. "Clinical and genetic heterogeneity of branching enzyme deficiency (glycogenosis type IV)." Neurology 63:1053-1058(2004) [PubMed] [Europe PMC] [Abstract]