Pathway maps

Galactose metabolism
Galactose metabolism

Object List (links open in MetaCore):

Glycerol, HXK2, PYGM, GYS2,, 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,,,, HXK1, Lactose synthase, PYGB, GYS1, Raffinose, Myo-Inositol, GALK2, PYGL, Stachyose,, D-Glucose,, D-Glucose,, alpha-(D)-Glucose- 6-phosphate, BGAL,, Glycogen phosphorylase, PGMU,, B4GT1, Melibiose, LPH,, LALBA, Glycerol 1-alpha- D-galactoside, HXK3, GALK1,,, Allolactose,,, D-Sorbitol, B4GT2, UDP-D-glucose,,, Galactinol, AGAL,, Melibiitol,, Glycogen,


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].


  1. Dean KJ, Sung SS, Sweeley CC
    Purification and partial characterization of human liver alpha-galactosidase: is alpha-galactosidase B an alpha-N-acetylgalactosaminidase? Advances in experimental medicine and biology 1978;101:515-23
  2. Bishop DF, Calhoun DH, Bernstein HS, Hantzopoulos P, Quinn M, Desnick RJ
    Human alpha-galactosidase A: nucleotide sequence of a cDNA clone encoding the mature enzyme. Proceedings of the National Academy of Sciences of the United States of America 1986 Jul;83(13):4859-63
  3. Ishii S, Kase R, Sakuraba H, Fujita S, Sugimoto M, Tomita K, Semba T, Suzuki Y
    Human alpha-galactosidase gene expression: significance of two peptide regions encoded by exons 1-2 and 6. Biochimica et biophysica acta 1994 Feb 16;1204(2):265-70
  4. Garman SC, Garboczi DN
    The molecular defect leading to Fabry disease: structure of human alpha-galactosidase. Journal of molecular biology 2004 Mar 19;337(2):319-35
  5. Naumov DG
    [Phylogenetic analysis of alpha-galactosidases of the GH27 family] Molekuliarnaia biologiia 2004 May-Jun;38(3):463-76
  6. Kanekura T, Fukushige T, Kanda A, Tsuyama S, Murata F, Sakuraba H, Kanzaki T
    Immunoelectron-microscopic detection of globotriaosylceramide accumulated in the skin of patients with Fabry disease. The British journal of dermatology 2005 Sep;153(3):544-8
  7. Sakuraba H, Murata-Ohsawa M, Kawashima I, Tajima Y, Kotani M, Ohshima T, Chiba Y, Takashiba M, Jigami Y, Fukushige T, Kanzaki T, Itoh K
    Comparison of the effects of agalsidase alfa and agalsidase beta on cultured human Fabry fibroblasts and Fabry mice. Journal of human genetics 2006;51(3):180-8
  8. Yagi F, Eckhardt AE, Goldstein IJ
    Glycosidases of Ehrlich ascites tumor cells and ascitic fluid--purification and substrate specificity of alpha-N-acetylgalactosaminidase and alpha-galactosidase: comparison with coffee bean alpha-galactosidase. Archives of biochemistry and biophysics 1990 Jul;280(1):61-7
  9. Dean KJ, Sweeley CC
    Studies on human liver alpha-galactosidases. II. Purification and enzymatic properties of alpha-galactosidase B (alpha-N-acetylgalactosaminidase). The Journal of biological chemistry 1979 Oct 25;254(20):10001-5
  10. Dean KJ, Sweeley CC
    Studies on human liver alpha-galactosidases. III. Partial characterization of carbohydrate-binding specificities. The Journal of biological chemistry 1979 Oct 25;254(20):10006-10
  11. Naim HY, Lentze MJ
    Impact of O-glycosylation on the function of human intestinal lactase-phlorizin hydrolase. Characterization of glycoforms varying in enzyme activity and localization of O-glycoside addition. The Journal of biological chemistry 1992 Dec 15;267(35):25494-504
  12. Buller HA, Rings EH, Montgomery RK, Grand RJ
    Clinical aspects of lactose intolerance in children and adults. Scandinavian journal of gastroenterology. Supplement. 1991;188:73-80
  13. Naim HY, Lacey SW, Sambrook JF, Gething MJ
    Expression of a full-length cDNA coding for human intestinal lactase-phlorizin hydrolase reveals an uncleaved, enzymatically active, and transport-competent protein. The Journal of biological chemistry 1991 Jul 5;266(19):12313-20
  14. Lean ME
    How does sibutramine work? International journal of obesity and related metabolic disorders : journal of the International Association for the Study of Obesity 2001 Dec;25 Suppl 4:S8-11
  15. Bodlaj G, Stocher M, Hufnagl P, Hubmann R, Biesenbach G, Stekel H, Berg J
    Genotyping of the lactase-phlorizin hydrolase -13910 polymorphism by LightCycler PCR and implications for the diagnosis of lactose intolerance. Clinical chemistry 2006 Jan;52(1):148-51
  16. Lee RT, Peterson CL, Calman AF, Herskowitz I, O'Donnell JJ
    Cloning of a human galactokinase gene (GK2) on chromosome 15 by complementation in yeast. Proceedings of the National Academy of Sciences of the United States of America 1992 Nov 15;89(22):10887-91
  17. Ai Y, Basu M, Bergsma DJ, Stambolian D
    Comparison of the enzymatic activities of human galactokinase GALK1 and a related human galactokinase protein GK2. Biochemical and biophysical research communications 1995 Jul 17;212(2):687-91
  18. Pastuszak I, O'Donnell J, Elbein AD
    Identification of the GalNAc kinase amino acid sequence. The Journal of biological chemistry 1996 Sep 27;271(39):23653-6
  19. Thoden JB, Holden HM
    The molecular architecture of human N-acetylgalactosamine kinase. The Journal of biological chemistry 2005 Sep 23;280(38):32784-91
  20. Soni T, Brivet M, Moatti N, Lemonnier A
    The Philadelphia variant of galactokinase in human erythrocytes: physicochemical and catalytic properties. Clinica chimica acta; international journal of clinical chemistry 1988 Jun 30;175(1):97-106
  21. Timson DJ, Reece RJ
    Functional analysis of disease-causing mutations in human galactokinase. European journal of biochemistry / FEBS 2003 Apr;270(8):1767-74
  22. Timson DJ, Reece RJ
    Sugar recognition by human galactokinase. BMC biochemistry [electronic resource]. 2003 Nov 4;4:16
  23. Thoden JB, Timson DJ, Reece RJ, Holden HM
    Molecular structure of human galactokinase: implications for type II galactosemia. The Journal of biological chemistry 2005 Mar 11;280(10):9662-70
  24. Shin-Buhring Y, Osang M, Ziegler R, Schaub J
    A method for galactose-1-phosphate uridyltransferase assay and the separation of its isozymes by DEAE-cellulose column chromatography. Clinica chimica acta; international journal of clinical chemistry 1976 Aug 2;70(3):371-7
  25. Seyrantepe V, Ozguc M, Coskun T, Ozalp I, Reichardt JK
    Identification of mutations in the galactose-1-phosphate uridyltransferase (GALT) gene in 16 Turkish patients with galactosemia, including a novel mutation of F294Y. Mutation in brief no. 235. Online. Human mutation 1999;13(4):339
  26. Goodman MT, Wu AH, Tung KH, McDuffie K, Cramer DW, Wilkens LR, Terada K, Reichardt JK, Ng WG
    Association of galactose-1-phosphate uridyltransferase activity and N314D genotype with the risk of ovarian cancer. American journal of epidemiology 2002 Oct 15;156(8):693-701
  27. Lai K, Langley SD, Khwaja FW, Schmitt EW, Elsas LJ
    GALT deficiency causes UDP-hexose deficit in human galactosemic cells. Glycobiology 2003 Apr;13(4):285-94
  28. Fung WL, Risch H, McLaughlin J, Rosen B, Cole D, Vesprini D, Narod SA
    The N314D polymorphism of galactose-1-phosphate uridyl transferase does not modify the risk of ovarian cancer. Cancer epidemiology, biomarkers & prevention : a publication of the American Association for Cancer Research, cosponsored by the American Society of Preventive Oncology 2003 Jul;12(7):678-80
  29. Holden HM, Rayment I, Thoden JB
    Structure and function of enzymes of the Leloir pathway for galactose metabolism. The Journal of biological chemistry 2003 Nov 7;278(45):43885-8
  30. Tedokon M, Suzuki K, Kayamori Y, Fujita S, Katayama Y
    Enzymatic assay of inorganic phosphate with use of sucrose phosphorylase and phosphoglucomutase. Clinical chemistry 1992 Apr;38(4):512-5
  31. Gloria-Bottini F, Lucarini N, Palmarino R, La Torre M, Nicotra M, Borgiani P, Cosmi E, Bottini E
    Phosphoglucomutase genetic polymorphism of newborns. American journal of human biology : the official journal of the Human Biology Council 2001 Jan-Feb;13(1):9-14
  32. Takahashi N, Omine H, Kaneko J, Miura A, Satoh C
    Identification of base substitutions in ten types of rare variants of phosphoglucomutase-1 (PGM1) encountered in Japanese. Human biology; an international record of research 2001 Oct;73(5):755-62
  33. Pang H, Koda Y, Soejima M, Kimura H
    Identification of human phosphoglucomutase 3 (PGM3) as N-acetylglucosamine-phosphate mutase (AGM1). Annals of human genetics 2002 Mar;66(Pt 2):139-44
  34. Gururaj A, Barnes CJ, Vadlamudi RK, Kumar R
    Regulation of phosphoglucomutase 1 phosphorylation and activity by a signaling kinase. Oncogene 2004 Oct 21;23(49):8118-27
  35. Ramakrishnan B, Boeggeman E, Qasba PK
    Beta-1,4-galactosyltransferase and lactose synthase: molecular mechanical devices. Biochemical and biophysical research communications 2002 Mar 15;291(5):1113-8
  36. Jackson JG, Janszen DB, Lonnerdal B, Lien EL, Pramuk KP, Kuhlman CF
    A multinational study of alpha-lactalbumin concentrations in human milk. The Journal of nutritional biochemistry. 2004 Sep;15(9):517-21
  37. Zhu X, Jiang J, Shen H, Wang H, Zong H, Li Z, Yang Y, Niu Z, Liu W, Chen X, Hu Y, Gu J
    Elevated beta1,4-galactosyltransferase I in highly metastatic human lung cancer cells. Identification of E1AF as important transcription activator. The Journal of biological chemistry 2005 Apr 1;280(13):12503-16
  38. Garcia-Vallejo JJ, van Dijk W, van Die I, Gringhuis SI
    Tumor necrosis factor-alpha up-regulates the expression of beta1,4-galactosyltransferase I in primary human endothelial cells by mRNA stabilization. The Journal of biological chemistry 2005 Apr 1;280(13):12676-82
  39. Ramakrishnan B, Ramasamy V, Qasba PK
    Structural snapshots of beta-1,4-galactosyltransferase-I along the kinetic pathway. Journal of molecular biology 2006 Apr 14;357(5):1619-33
  40. Shaper NL, Charron M, Lo NW, Shaper JH
    Beta1,4-galactosyltransferase and lactose biosynthesis: recruitment of a housekeeping gene from the nonmammalian vertebrate gene pool for a mammary gland specific function. Journal of mammary gland biology and neoplasia 1998 Jul;3(3):315-24
  41. Amado M, Almeida R, Schwientek T, Clausen H
    Identification and characterization of large galactosyltransferase gene families: galactosyltransferases for all functions. Biochimica et biophysica acta 1999 Dec 6;1473(1):35-53
  42. Hansske B, Thiel C, Lubke T, Hasilik M, Honing S, Peters V, Heidemann PH, Hoffmann GF, Berger EG, von Figura K, Korner C
    Deficiency of UDP-galactose:N-acetylglucosamine beta-1,4-galactosyltransferase I causes the congenital disorder of glycosylation type IId. The Journal of clinical investigation 2002 Mar;109(6):725-33
  43. Ramakrishnan B, Balaji PV, Qasba PK
    Crystal structure of beta1,4-galactosyltransferase complex with UDP-Gal reveals an oligosaccharide acceptor binding site. Journal of molecular biology 2002 Apr 26;318(2):491-502
  44. Saitoh E, Aoki D, Susumu N, Udagawa Y, Nozawa S
    Galactosyltransferase associated with tumor in patients with ovarian cancer: factors involved in elevation of serum galactosyltransferase. International journal of oncology 2003 Aug;23(2):303-10
  45. Wohlers TM, Christacos NC, Harreman MT, Fridovich-Keil JL
    Identification and characterization of a mutation, in the human UDP-galactose-4-epimerase gene, associated with generalized epimerase-deficiency galactosemia. American journal of human genetics 1999 Feb;64(2):462-70
  46. Thoden JB, Wohlers TM, Fridovich-Keil JL, Holden HM
    Human UDP-galactose 4-epimerase. Accommodation of UDP-N-acetylglucosamine within the active site. The Journal of biological chemistry 2001 May 4;276(18):15131-6
  47. Schulz JM, Watson AL, Sanders R, Ross KL, Thoden JB, Holden HM, Fridovich-Keil JL
    Determinants of function and substrate specificity in human UDP-galactose 4'-epimerase. The Journal of biological chemistry 2004 Jul 30;279(31):32796-803
  48. Timson DJ
    Functional analysis of disease-causing mutations in human UDP-galactose 4-epimerase. The FEBS journal 2005 Dec;272(23):6170-7
  49. Openo KK, Schulz JM, Vargas CA, Orton CS, Epstein MP, Schnur RE, Scaglia F, Berry GT, Gottesman GS, Ficicioglu C, Slonim AE, Schroer RJ, Yu C, Rangel VE, Keenan J, Lamance K, Fridovich-Keil JL
    Epimerase-deficiency galactosemia is not a binary condition. American journal of human genetics 2006 Jan;78(1):89-102
  50. Westphal SA, Nuttall FQ
    Comparative characterization of human and rat liver glycogen synthase. Archives of biochemistry and biophysics 1992 Feb 1;292(2):479-86
  51. Ferrer JC, Favre C, Gomis RR, Fernandez-Novell JM, Garcia-Rocha M, de la Iglesia N, Cid E, Guinovart JJ
    Control of glycogen deposition. FEBS letters 2003 Jul 3;546(1):127-32
  52. Cid E, Cifuentes D, Baque S, Ferrer JC, Guinovart JJ
    Determinants of the nucleocytoplasmic shuttling of muscle glycogen synthase. The FEBS journal 2005 Jun;272(12):3197-213
  53. Weinstein DA, Correia CE, Saunders AC, Wolfsdorf JI
    Hepatic glycogen synthase deficiency: an infrequently recognized cause of ketotic hypoglycemia. Molecular genetics and metabolism 2006 Apr;87(4):284-8
  54. Browner MF, Nakano K, Bang AG, Fletterick RJ
    Human muscle glycogen synthase cDNA sequence: a negatively charged protein with an asymmetric charge distribution. Proceedings of the National Academy of Sciences of the United States of America 1989 Mar;86(5):1443-7
  55. Orho M, Nikula-Ijas P, Schalin-Jantti C, Permutt MA, Groop LC
    Isolation and characterization of the human muscle glycogen synthase gene. Diabetes 1995 Sep;44(9):1099-105
  56. Peng HL, Chang HY
    Cloning of a human liver UDP-glucose pyrophosphorylase cDNA by complementation of the bacterial galU mutation. FEBS letters 1993 Aug 23;329(1-2):153-8
  57. Su X, Schuler L, Shapiro S
    Cloning and characterization of a glycogen synthase cDNA from human endometrium. The Journal of steroid biochemistry and molecular biology 1996 Dec;59(5-6):459-65
  58. Nielsen JN, Richter EA
    Regulation of glycogen synthase in skeletal muscle during exercise. Acta physiologica Scandinavica. 2003 Aug;178(4):309-19
  59. Motoyama K, Emoto M, Tahara H, Komatsu M, Shoji T, Inaba M, Nishizawa Y
    Association of muscle glycogen synthase polymorphism with insulin resistance in type 2 diabetic patients. Metabolism: clinical and experimental 2003 Jul;52(7):895-9
  60. Browner MF, Fletterick RJ
    Phosphorylase: a biological transducer. Trends in biochemical sciences 1992 Feb;17(2):66-71
  61. Newgard CB, Littman DR, van Genderen C, Smith M, Fletterick RJ
    Human brain glycogen phosphorylase. Cloning, sequence analysis, chromosomal mapping, tissue expression, and comparison with the human liver and muscle isozymes. The Journal of biological chemistry 1988 Mar 15;263(8):3850-7
  62. Burwinkel B, Bakker HD, Herschkovitz E, Moses SW, Shin YS, Kilimann MW
    Mutations in the liver glycogen phosphorylase gene (PYGL) underlying glycogenosis type VI. American journal of human genetics 1998 Apr;62(4):785-91
  63. Chang S, Rosenberg MJ, Morton H, Francomano CA, Biesecker LG
    Identification of a mutation in liver glycogen phosphorylase in glycogen storage disease type VI. Human molecular genetics 1998 May;7(5):865-70
  64. Kubisch C, Wicklein EM, Jentsch TJ
    Molecular diagnosis of McArdle disease: revised genomic structure of the myophosphorylase gene and identification of a novel mutation. Human mutation 1998;12(1):27-32
  65. Rath VL, Ammirati M, Danley DE, Ekstrom JL, Gibbs EM, Hynes TR, Mathiowetz AM, McPherson RK, Olson TV, Treadway JL, Hoover DJ
    Human liver glycogen phosphorylase inhibitors bind at a new allosteric site. Chemistry & biology 2000 Sep;7(9):677-82
  66. Ercan-Fang N, Gannon MC, Rath VL, Treadway JL, Taylor MR, Nuttall FQ
    Integrated effects of multiple modulators on human liver glycogen phosphorylase a. American journal of physiology. Endocrinology and metabolism. 2002 Jul;283(1):E29-37
  67. Ekstrom JL, Pauly TA, Carty MD, Soeller WC, Culp J, Danley DE, Hoover DJ, Treadway JL, Gibbs EM, Fletterick RJ, Day YS, Myszka DG, Rath VL
    Structure-activity analysis of the purine binding site of human liver glycogen phosphorylase. Chemistry & biology 2002 Aug;9(8):915-24
  68. Rake JP, Visser G, Labrune P, Leonard JV, Ullrich K, Smit GP
    Glycogen storage disease type I: diagnosis, management, clinical course and outcome. Results of the European Study on Glycogen Storage Disease Type I (ESGSD I). European journal of pediatrics 2002 Oct;161 Suppl 1:S20-34
  69. Bandsma RH, Smit GP, Kuipers F
    Disturbed lipid metabolism in glycogen storage disease type 1. European journal of pediatrics 2002 Oct;161 Suppl 1:S65-9
  70. Mortellaro C, Garagiola U, Carbone V, Cerutti F, Marci V, Bonda PL
    Unusual oral manifestations and evolution in glycogen storage disease type Ib. The Journal of craniofacial surgery 2005 Jan;16(1):45-52
  71. Han SH, Ki CS, Lee JE, Hong YJ, Son BK, Lee KH, Choe YH, Lee SY, Kim JW
    A novel mutation (A148V) in the glucose 6-phosphate translocase (SLC37A4) gene in a Korean patient with glycogen storage disease type 1b. Journal of Korean medical science 2005 Jun;20(3):499-501
  72. Magnani M, Stocchi V, Serafini G, Chiarantini L, Fornaini G
    Purification, properties, and evidence for two subtypes of human placenta hexokinase type I. Archives of biochemistry and biophysics 1988 Jan;260(1):388-99
  73. Magnani M, Serafini G, Bianchi M, Casabianca A, Stocchi V
    Human hexokinase type I microheterogeneity is due to different amino-terminal sequences. The Journal of biological chemistry 1991 Jan 5;266(1):502-5
  74. Bianchi M, Magnani M
    Hexokinase mutations that produce nonspherocytic hemolytic anemia. Blood cells, molecules & diseases 1995;21(1):2-8
  75. Murakami K, Piomelli S
    Identification of the cDNA for human red blood cell-specific hexokinase isozyme. Blood 1997 Feb 1;89(3):762-6
  76. Lowes W, Walker M, Alberti KG, Agius L
    Hexokinase isoenzymes in normal and cirrhotic human liver: suppression of glucokinase in cirrhosis. Biochimica et biophysica acta 1998 Jan 8;1379(1):134-42
  77. Aleshin AE, Zeng C, Bourenkov GP, Bartunik HD, Fromm HJ, Honzatko RB
    The mechanism of regulation of hexokinase: new insights from the crystal structure of recombinant human brain hexokinase complexed with glucose and glucose-6-phosphate. Structure (London, England : 1993) 1998 Jan 15;6(1):39-50
  78. Bianchi M, Serafini G, Bartolucci E, Palma F, Magnani M
    Expression, purification, and characterization of a recombinant erythroid-specific hexokinase isozyme. Blood cells, molecules & diseases 1998 Dec;24(4):401-11
  79. Aleshin AE, Zeng C, Bartunik HD, Fromm HJ, Honzatko RB
    Regulation of hexokinase I: crystal structure of recombinant human brain hexokinase complexed with glucose and phosphate. Journal of molecular biology 1998 Sep 18;282(2):345-57
  80. Laakso M, Malkki M, Deeb SS
    Amino acid substitutions in hexokinase II among patients with NIDDM. Diabetes 1995 Mar;44(3):330-4
  81. Vidal-Puig A, Printz RL, Stratton IM, Granner DK, Moller DE
    Analysis of the hexokinase II gene in subjects with insulin resistance and NIDDM and detection of a Gln142-->His substitution. Diabetes 1995 Mar;44(3):340-6
  82. Echwald SM, Bjorbaek C, Hansen T, Clausen JO, Vestergaard H, Zierath JR, Printz RL, Granner DK, Pedersen O
    Identification of four amino acid substitutions in hexokinase II and studies of relationships to NIDDM, glucose effectiveness, and insulin sensitivity. Diabetes 1995 Mar;44(3):347-53
  83. Vestergaard H, Bjorbaek C, Hansen T, Larsen FS, Granner DK, Pedersen O
    Impaired activity and gene expression of hexokinase II in muscle from non-insulin-dependent diabetes mellitus patients. The Journal of clinical investigation 1995 Dec;96(6):2639-45
  84. Lehto M, Huang X, Davis EM, Le Beau MM, Laurila E, Eriksson KF, Bell GI, Groop L
    Human hexokinase II gene: exon-intron organization, mutation screening in NIDDM, and its relationship to muscle hexokinase activity. Diabetologia 1995 Dec;38(12):1466-74
  85. Furuta H, Nishi S, Le Beau MM, Fernald AA, Yano H, Bell GI
    Sequence of human hexokinase III cDNA and assignment of the human hexokinase III gene (HK3) to chromosome band 5q35.2 by fluorescence in situ hybridization. Genomics 1996 Aug 15;36(1):206-9
  86. Sebastian S, Edassery S, Wilson JE
    The human gene for the type III isozyme of hexokinase: structure, basal promoter, and evolution. Archives of biochemistry and biophysics 2001 Nov 1;395(1):113-20
  87. Koranyi LI, Tanizawa Y, Welling CM, Rabin DU, Permutt MA
    Human islet glucokinase gene. Isolation and sequence analysis of full-length cDNA. Diabetes 1992 Jul;41(7):807-11
  88. Tanizawa Y, Matsutani A, Chiu KC, Permutt MA
    Human glucokinase gene: isolation, structural characterization, and identification of a microsatellite repeat polymorphism. Molecular endocrinology (Baltimore, Md.) 1992 Jul;6(7):1070-81
  89. Nishi S, Stoffel M, Xiang K, Shows TB, Bell GI, Takeda J
    Human pancreatic beta-cell glucokinase: cDNA sequence and localization of the polymorphic gene to chromosome 7, band p 13. Diabetologia 1992 Aug;35(8):743-7
  90. Xu LZ, Harrison RW, Weber IT, Pilkis SJ
    Human beta-cell glucokinase. Dual role of Ser-151 in catalysis and hexose affinity. The Journal of biological chemistry 1995 Apr 28;270(17):9939-46
  91. St Charles R, Harrison RW, Bell GI, Pilkis SJ, Weber IT
    Molecular model of human beta-cell glucokinase built by analogy to the crystal structure of yeast hexokinase B. Diabetes 1994 Jun;43(6):784-91
  92. Agius L, Peak M
    Intracellular binding of glucokinase in hepatocytes and translocation by glucose, fructose and insulin. The Biochemical journal 1993 Dec 15;296 ( Pt 3):785-96
  93. Takeda J, Gidh-Jain M, Xu LZ, Froguel P, Velho G, Vaxillaire M, Cohen D, Shimada F, Makino H, Nishi S
    Structure/function studies of human beta-cell glucokinase. Enzymatic properties of a sequence polymorphism, mutations associated with diabetes, and other site-directed mutants. The Journal of biological chemistry 1993 Jul 15;268(20):15200-4
  94. Iynedjian PB
    Mammalian glucokinase and its gene. The Biochemical journal 1993 Jul 1;293 ( Pt 1):1-13
  95. Xu LZ, Weber IT, Harrison RW, Gidh-Jain M, Pilkis SJ
    Sugar specificity of human beta-cell glucokinase: correlation of molecular models with kinetic measurements. Biochemistry 1995 May 9;34(18):6083-92
  96. Downward J
    Cell biology: metabolism meets death. Nature 2003 Aug 21;424(6951):896-7
  97. Baltrusch S, Francini F, Lenzen S, Tiedge M
    Interaction of glucokinase with the liver regulatory protein is conferred by leucine-asparagine motifs of the enzyme. Diabetes 2005 Oct;54(10):2829-37