GBE1
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Official Full Name
glucan (1,4-alpha-), branching enzyme 1 -
Overview
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 Andersens disease). -
Synonyms
GBE1;glucan (1,4-alpha-), branching enzyme 1;1,4-alpha-glucan-branching enzyme;Andersen disease;glycogen branching enzyme;glycogen storage disease type IV;1;1,4 alpha glucan branching enzyme;4-alpha-glucan-branching enzyme;amylo (1,4 to 1,6) transglucosidase;amylo (1,4 to 1,6) transglycosylase;Brancher enzyme;GBE 1;GBE;gGlucan (1,4 alpha ), branching enzyme 1;GLGB_HUMAN;Glucan (1,4 alpha) branching enzyme;Glycogen-branching enzyme;OTTHUMP00000213788;OTTHUMP00000213833;amylo-(1,4 to 1,6) transglucosidase;amylo-(1,4 to 1,6) transglycosylase
Recombinant Proteins
- Rhesus macaque
- Human
- Mouse
- Mammalian Cell
- Wheat Germ
- HEK293
- In Vitro Cell Free System
- Mammalian cells
- HEK293T
- His
- Non
- Myc&DDK
- His&Fc&Avi
- Flag
- GST
Involved Pathway
GBE1 involved in several pathways and played different roles in them. We selected most pathways GBE1 participated on our site, such as Starch and sucrose metabolism,Metabolic pathways, which may be useful for your reference. Also, other proteins which involved in the same pathway with GBE1 were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.
| Pathway Name | Pathway Related Protein |
|---|---|
| Metabolic pathways | ALPI,GUK1B,RRM1,CKB,CYP2C70,NOS2,NDST4,AHCYL2,COQ6,POLA1 |
| Starch and sucrose metabolism | G6PC,UGT1A9,AMY2A4,UGDH,GPIB,GPIA,AMY2A,GAA,UGT1A7C,AMY2A2 |
Protein Function
GBE1 has several biochemical functions, for example, 1,4-alpha-glucan branching enzyme activity,cation binding,hydrolase activity, hydrolyzing O-glycosyl compounds. Some of the functions are cooperated with other proteins, some of the functions could acted by GBE1 itself. We selected most functions GBE1 had, and list some proteins which have the same functions with GBE1. You can find most of the proteins on our site.
| Function | Related Protein |
|---|---|
| cation binding | SLC3A1,PPM1E,SLC3A2B,Amy1,SLC3A2,PPM1AA,PPM1DA,PPM1BB,PPM1LB,PPM1DB |
| hydrolase activity, hydrolyzing O-glycosyl compounds | KLB,CHIA.1,CHIA.2,Chil3,LCTLA,LCTLB,ABHD10,CHIA.4,GNE,CHIA.3 |
Interacting Protein
GBE1 has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with GBE1 here. Most of them are supplied by our site. Hope this information will be useful for your research of GBE1.
TRDMT1;PGD;EPHB3;PRMT6;1-phosphatidyl-1d-myo-inositol 4,5-bisphosphate;PRICKLE3;OAZ1;Kctd5;KIFAP3;Cdc20;SETD4;VAPA;Rhoa
Resources
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References
- Zmasek, CM; Godzik, A; et al. Phylogenomic analysis of glycogen branching and debranching enzymatic duo. BMC EVOLUTIONARY BIOLOGY 14:-(2014).
- Bit-Ivan, EN; Lee, KH; et al. Adult polyglucosan body disease with GBE1 haploinsufficiency and concomitant frontotemporal lobar degeneration. NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY 40:778-782(2014).
