Pathway maps

Galactose metabolism
Galactose metabolism

Object List (links open in MetaCore):

Glycerol, HXK2, PYGM, GYS2, 2.4.1.1, Lactose-6- phosphate, Lactose, GALE, HXK4, G6PT, D-Galactose, alpha-(D)-Glucose 1-phosphate, UDP-D-galactose, GALT, alpha-(D)-Galactose 1-phosphate, D-Mannose, 2.7.1.1, 3.2.1.22, 3.1.3.9, HXK1, Lactose synthase, PYGB, GYS1, Raffinose, Myo-Inositol, GALK2, PYGL, Stachyose, 2.4.1.11, D-Glucose, 5.3.1.2, D-Glucose, 2.4.1.22, alpha-(D)-Glucose- 6-phosphate, BGAL, 2.7.1.6, Glycogen phosphorylase, PGMU, 3.2.1.22, B4GT1, Melibiose, LPH, 3.2.1.108, LALBA, Glycerol 1-alpha- D-galactoside, HXK3, GALK1, 3.2.1.22, 3.2.1.22, Allolactose, 3.2.1.22, 5.4.2.2, D-Sorbitol, B4GT2, UDP-D-glucose, 2.7.7.12, 3.2.1.22, Galactinol, AGAL, 3.2.1.22, Melibiitol, 2.7.1.2, Glycogen, 3.2.1.23

Description

Galactose metabolism

Congenital error in metabolism of D-Galactose characterized by inability of an organism to normally metabolize galactose and lactose is called as a galactose diabetes or Galactosamia.

D-Galactose is a constituent of oligosaccharides and is produced by hydrolysis of Melibiose [1], [2], [3], [4], [5], [6], [7], Raffinose [4], [6], [7], Stachyose [4], [6], [7], Allolactose [1], [8], [9], [10], [2], [4], [6], [7], Glycerol 1-alpha-D-galactoside [4], [6], [7], Melibitol [4], [5], [6], [7], Galactinol [4], [5], [6], [7] by Galactosidase, alpha ( AGAL ), or by hydrolysis of Lactose by Lactase ( LPH ) [11], [12], [13], [14]. [15].

Alpha-(D)-Galactose 1-phosphate if formed by transfer of the phosphate moiety from ATP to D-Galactose catalyzed by Galactokinase 2 ( GALK2 ) [16], [17], [18], [19] and Galactokinase 1 ( GALK1 ) [20], [17], [21], [22], [23], [19].

Subsequent transformation of the alpha-(D)-Galactose 1-phosphate to alpha-D-Glucose 1-phosphate and UDP-D-galactose is catalyzed by Galactose-1-phosphate uridylyltransferase ( GALT ) [24], [25], [26], [27], [28], [29]. Both products participate in the further transformations. Phosphoglucomutase 1 ( PGMU ) then catalyzes formation of the alpha-(D)-Glucose-6-phosphate from alpha-D-Glucose 1-phosphate [30], [31], [32], [33], [34]. UDP-D-galactose is transformed further in the two subsequent reactions. In the first reaction the formation of Lactose by group of enzymes occurred: UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1 ( Lactose synthase) [35], [36], [37], [38], [39] and by UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2 ( B4GT2 ) [40], [41], [42], [35], [43], [44], [39]. In the second reaction UDP-D-galactose is transformed to UDP-D-glucose by UDP-galactose-4-epimerase ( GALE ) [45], [46], [29], [47], [48], [49]. UDP-D-glucose participates in formation of Glycogen catalyzed by two enzymes, glycogen synthase 2 (liver) ( GYS2 ) [50], [51], [52], [53] and by glycogen synthase 1 (muscle) ( GYS1 ) [54], [55], [56], [57], [51], [58], [59], [52]. Glycogen is the substrate of one more reaction of formation alpha-D-Glucose 1-phosphate catalyzed by Glycogen phosphorylase [60], [61], [62], [63], [64], [65], [66], [67].

Apha-D-Glucose 6-phosphate also participates in glycolysis and gluconeogenesis. Hydrolysis of Apha-D-Glucose 6-phosphate to D-Glucose is catalyzed by Glucose-6-phosphatase, catalytic subunit ( G6PT ) [68], [69], [70], [71]. This reaction can proceed also in the opposite direction under catalysis of other enzymes, Hexokinase 1 ( HXK1 ) [72], [73], [74], [75], [76], [77], [78], [79], Hexokinase-2 ( HXK2 ) [80], [81], [82], [83], [84], [76], Hexokinase 3 (white cell) ( HXK3 ) [85], [76], [86] and Glucokinase (hexokinase 4) ( HXK4 ) [87], [88], [89], [90], [91], [92], [93], [94], [95], [76], [96], [97].

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