产品名称
Glycogen Synthase 1 Rabbit Polyclonal Antibody
别名
GYS1; GYS; Glycogen [starch] synthase; muscle
蛋白名称
Glycogen [starch] synthase muscle
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=2997
Human Swissprot No.
P13807
Human Swissprot Link
http://www.uniprot.org/uniprotkb/P13807/entry
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=14936
Mouse Swissprot No.
Q9Z1E4
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q9Z1E4
Rat Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=690987
Rat Swissprot Link
http://www.uniprot.org/uniprot/A2RRU1
免疫原
The antiserum was produced against synthesized peptide derived from human Glycogen Synthase. AA range:621-670
特异性
Glycogen Synthase 1 Polyclonal Antibody detects endogenous levels of Glycogen Synthase 1 protein.
稀释度
WB 1:500 - 1:2000. ELISA: 1:10000.. IF 1:50-200
宿主
Polyclonal, Rabbit,IgG
背景介绍
The protein encoded by this gene catalyzes the addition of glucose monomers to the growing glycogen molecule through the formation of alpha-1,4-glycoside linkages. Mutations in this gene are associated with muscle glycogen storage disease. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Sep 2009],
组织表达
Endometrium,Heart,Kidney,Lymph,Muscle,Skin,
细胞定位
cytosol,membrane,inclusion body,
信号通路
Starch and sucrose metabolism;Insulin_Receptor;
功能
catalytic activity:UDP-glucose ((1->4)-alpha-D-glucosyl)(n) = UDP + ((1->4)-alpha-D-glucosyl)(n+1).,disease:Defects in GYS1 are the cause of muscle glycogen storage disease type 0 (GSD0b) [MIM:611556]; also called muscle glycogen synthase deficiency. GSD0 is a metabolic disorder characterized by fasting hypoglycemia presenting in infancy or early childhood. The role of muscle glycogen is to provide critical energy during bursts of activity and sustained muscle work.,enzyme regulation:Allosteric activation by glucose-6-phosphate. Phosphorylation reduces the activity towards UDP-glucose. When in the non-phosphorylated state, glycogen synthase does not require glucose-6-phosphate as an allosteric activator; when phosphorylated it does.,function:Transfers the glycosyl residue from UDP-Glc to the non-reducing end of alpha-1,4-glucan.,pathway:Glycan biosynthesis; glycogen biosynthesis.,similarity:Belongs to the glycosyltransferase 3 family.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
