信号通路: Fructose and mannose metabolism;Amino sugar and nucleotide sugar metabolism;
功能: catalytic activity:D-mannose 6-phosphate = D-fructose 6-phosphate.,cofactor:Binds 1 zinc ion per subunit.,disease:Defects in MPI are the cause of congenital disorder of glycosylation type 1B (CDG1B) [MIM:602579]; also known as carbohydrate-deficient glycoprotein syndrome type Ib (CDGS1B). Congenital disorders of glycosylation are metabolic deficiencies in glycoprotein biosynthesis that usually cause severe mental and psychomotor retardation. They are characterized by under-glycosylated serum glycoproteins. CDG1B is clinically characterized by protein-losing enteropathy.,function:Involved in the synthesis of the GDP-mannose and dolichol-phosphate-mannose required for a number of critical mannosyl transfer reactions.,pathway:Nucleotide-sugar biosynthesis; GDP-D-mannose biosynthesis; alpha-D-mannose 1-phosphate from D-fructose 6-phosphate: step 1/2.,similarity:Belongs to the mannose-6-phosphate isomerase type 1 family.,tissue specificity:Expressed in all tissues, but more abundant in heart, brain and skeletal muscle.,
相关产品: RS0001,RS0002,YM3028,YM3029
细胞定位: Cytoplasm .
组织表达: Expressed in all tissues, but more abundant in heart, brain and skeletal muscle.
信号通路: Fructose and mannose metabolism;Amino sugar and nucleotide sugar metabolism;
功能: catalytic activity:D-mannose 6-phosphate = D-fructose 6-phosphate.,cofactor:Binds 1 zinc ion per subunit.,disease:Defects in MPI are the cause of congenital disorder of glycosylation type 1B (CDG1B) [MIM:602579]; also known as carbohydrate-deficient glycoprotein syndrome type Ib (CDGS1B). Congenital disorders of glycosylation are metabolic deficiencies in glycoprotein biosynthesis that usually cause severe mental and psychomotor retardation. They are characterized by under-glycosylated serum glycoproteins. CDG1B is clinically characterized by protein-losing enteropathy.,function:Involved in the synthesis of the GDP-mannose and dolichol-phosphate-mannose required for a number of critical mannosyl transfer reactions.,pathway:Nucleotide-sugar biosynthesis; GDP-D-mannose biosynthesis; alpha-D-mannose 1-phosphate from D-fructose 6-phosphate: step 1/2.,similarity:Belongs to the mannose-6-phosphate isomerase type 1 family.,tissue specificity:Expressed in all tissues, but more abundant in heart, brain and skeletal muscle.,
相关产品: RS0001,RS0002,YM3028,YM3029
细胞定位: Cytoplasm .
组织表达: Expressed in all tissues, but more abundant in heart, brain and skeletal muscle.