免疫原
Purified recombinant fragment of human GCK expressed in E. Coli.
特异性
GCK Monoclonal Antibody detects endogenous levels of GCK protein.
组成
Ascitic fluid containing 0.03% sodium azide,0.5% BSA, 50%glycerol.
稀释比例
Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications.
纯化工艺
Affinity purification
其他名称
GCK; Glucokinase; Hexokinase type IV; HK IV; Hexokinase-4; HK4; Hexokinase-D
背景
Hexokinases phosphorylate glucose to produce glucose-6-phosphate, the first step in most glucose metabolism pathways. Alternative splicing of this gene results in three tissue-specific forms of glucokinase, one found in pancreatic islet beta cells and two found in liver. The protein localizes to the outer membrane of mitochondria. In contrast to other forms of hexokinase, this enzyme is not inhibited by its product glucose-6-phosphate but remains active while glucose is abundant. Mutations in this gene have been associated with non-insulin dependent diabetes mellitus (NIDDM), maturity onset diabetes of the young, type 2 (MODY2) and persistent hyperinsulinemic hypoglycemia of infancy (PHHI). [provided by RefSeq, Apr 2009],
功能
catalytic activity:ATP + D-glucose = ADP + D-glucose 6-phosphate.,disease:Defects in GCK are the cause of familial hyperinsulinemic hypoglycemia type 3 (HHF3) [MIM:602485]. HHF is the most common cause of persistent hypoglycemia in infancy. Unless early and aggressive intervention is undertaken, brain damage from recurrent episodes of hypoglycemia may occur.,disease:Defects in GCK are the cause of maturity onset diabetes of the young type 2 (MODY2) [MIM:125851]; also shortened MODY-2. MODY [MIM:606391] is a form of diabetes mellitus characterized by autosomal dominant transmission and early age of onset. Mutations in GCK result in mild chronic hyperglycemia due to reduced pancreatic beta cell responsiveness to glucose, decreased net accumulation of hepatic glycogen and increased hepatic gluconeogenesis following meals.,enzyme regulation:The use of alternative promoters apparently enables
