GBE1 polyclonal antibody (A01)
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Specification
Product Description
Mouse polyclonal antibody raised against a partial recombinant GBE1.
Immunogen
GBE1 (NP_000149, 605 a.a. ~ 702 a.a) partial recombinant protein with GST tag.
Sequence
SEKHEGNKIIAFERAGLLFIFNFHPSKSYTDYRVGTALPGKFKIVLDSDAAEYGGHQRLDHSTDFFSEAFEHNGRPYSLLVYIPSRVALILQNVDLPN
Host
Mouse
Reactivity
Human
Interspecies Antigen Sequence
Mouse (91); Rat (95)
Quality Control Testing
Antibody Reactive Against Recombinant Protein.
Western Blot detection against Immunogen (36.89 KDa) .
Storage Buffer
50 % glycerol
Storage Instruction
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
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Applications
Western Blot (Recombinant protein)
ELISA
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Gene Info — GBE1
Entrez GeneID
2632GeneBank Accession#
NM_000158Protein Accession#
NP_000149Gene Name
GBE1
Gene Alias
GBE
Gene Description
glucan (1,4-alpha-), branching enzyme 1
Gene Ontology
HyperlinkGene 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
Other Designations
amylo-(1,4 to 1,6) transglucosidase|amylo-(1,4 to 1,6) transglycosylase|glycogen branching enzyme|glycogen storage disease type IV
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Interactome
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Pathway
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Disease
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Publication Reference
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Muscle glycogen remodeling and glycogen phosphate metabolism following exhaustive exercise of wild type and laforin knockout mice.
Irimia JM, Tagliabracci VS, Meyer CM, Segvich DM, DePaoli-Roach AA, Roach PJ.
The Journal of Biological Chemistry 2015 Jul; 290(37):22686.
Application:WB, Mouse, Skeletal muscle.
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Polyglucosan neurotoxicity caused by glycogen branching enzyme deficiency can be reversed by inhibition of glycogen synthase.
Kakhlon O, Glickstein H, Feinstein N, Liu Y, Baba O, Terashima T, Akman HO, Dimauro S, Lossos A.
Journal of Neurochemistry 2013 Oct; 127(1):101.
Application:IF, WB-Tr, Human, Rat, Human lymphocytes, Primary neurons from E18 rat cortex.
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Muscle glycogen remodeling and glycogen phosphate metabolism following exhaustive exercise of wild type and laforin knockout mice.
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