New aspects of proinsulin physiology and pathophysiology

Journal of Pediatric Endocrinology and Metabolism

Volumn 13, Issue 3, 2000, Pages 229-239

  Steiner, Donald F ^a  

^a UNIVERSITY OF CHICAGO   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

INSULIN; PROINSULIN;

DIABETES MELLITUS; GENETIC DISORDER; HORMONE DETERMINATION; HUMAN; INSULIN SYNTHESIS; REVIEW; ROUGH ENDOPLASMIC RETICULUM;

EID: 0034070828     PISSN: 0334018X     EISSN: None     Source Type: Journal    
DOI: 10.1515/JPEM.2000.13.3.229     Document Type: Review

References (62)

1. Steiner DF, Bell GI, Tager HS. Chemistry and biosynthesis of pancreatic protein hormones. In: DeGroot L, ed. Endocrinology, Vol. 2. Philadelphia, PA: W.B. Saunders, 1995; 1296-1328.
2. Polonsky KS, O'Meara NM. Secretion and metabolism of insulin, proinsulin, and C-peptide. In: DeGroot LJ, ed. Endocrinology, Vol. 2. Philadelphia, PA: W.B. Saunders, 1995; 1354-1372.
3. Wareham NJ, Byrne CD, Williams R, Day NE, Hales CN. Fasting proinsulin concentrations predict the development of type 2 diabetes. Diabetes Care 1999; 22: 262-270.
4. Roder ME, Dinesen B, Hartling SG, Houssa P, Vestergaard H, Sodoyez-Goffaux F, Binder C. Intact proinsulin and β-cell function in lean and obese subjects with and without type 2 diabetes. Diabetes Care 1999; 22: 609-614.
5. Rouillé Y, Duguay S, Lund K, Furuta M, Gong Q, Lipkind G, Oliva A Jr, Chan S, Steiner D. Proteolytic processing mechanisms in the biosynthesis of neuroendocrine peptides: the subtilisin-like proprotein convertases. Front Neuroendocrinol 1995; 16: 322-361.
6. Chan S, Keim P, Steiner D. Cell-free synthesis of rat preproinsulins: characterization and partial amino acid sequence determination. Proc Natl Acad Sci USA 1976; 73: 1964-1968.
7. Patzelt C, Labrecque A, Duguid J, Carroll R, Keim P, Heinrikson R, Steiner D. Detection and kinetic behavior of preproinsulin in pancreatic islets. Proc Natl Acad Sci USA 1978; 75: 1260-1264.
8. Huang XF, Arvan P. Intracellular transport of proinsulin in pancreatic β-cells. J Biol Chem 1995; 270: 20417-20423.
9. Orci L, Ravazzola M, Amherdt M, Madsen O, Perrelet A, Vassali J-D, Anderson RG. Conversion of proinsulin to insulin occurs coordinately with acidification of maturing secretory vesicles. J Cell Biol 1986; 103: 2273-2281.
10. Rhodes CJ, Halban PA. The intracellular handling of insulin-related peptides in isolated pancreatic islets. Biochem J 1988; 251: 23-30.
11. 2+ sequestration in diabetic mouse islets of Langerhans. J Biol Chem 1994; 269: 18279-18282.
12. 2+ and G-proteins. Diabetes Rev 1996; 4: 276-297.
13. Zhou A, Webb G, Zhu X, Steiner DF. Proteolytic processing in the secretory pathway. J Biol Chem 1999; 274: 20745-20748.
14. Zhou A, Martin S, Lipkind G, LaMendola J, Steiner D. Regulatory roles of the P domain of the subtilisin-like prohormone convertases. J Biol Chem 1998; 273: 11107-11114.
15. Molloy S, Anderson E, Jean F, Thomas G. Bi-cycling the furin pathway: from TGN localization to pathogen activation and embryogenesis. Trends Cell Biol 1999; 9: 28-35.
16. Muller L, Zhu X, Lindberg I. Mechanism of the facilitation of PC2 maturation by 7B2: involvement in pro-PC2 transport and activation but not folding. J Cell Biol 1997; 139: 625-638.
17. Lamango NS, Apletalina E, Liu J, Lindberg I. The proteolytic maturation of prohormone convertase 2 (PC2) is a pH-driven process. Arch Biochem Biophys 1999; 362: 275-282.
18. Rhodes C, Lincoln B, Shoelson S. Preferential cleavage of des-31,32-proinsulin over intact proinsulin by the insulin secretory granule type 2 endopeptidase. J Biol Chem 1992; 267: 22719-22727.
19. Furuta M, Yano H, Zhou A, Rouillé Y, Holst J, Carroll R, Ravazzola M, Orci L, Furuta H, Steiner D. Defective prohormone processing and altered pancreatic islet morphology in mice lacking active SPC2. Proc Natl Acad Sci USA 1997; 94: 6646-6651.
20. Jackson R, Creemers J, Ohagi S, Raffin-Sanson M-L, Sanders L, Montague C, Hutton J, O'Rahilly S. Obesity and impaired prohormone processing associated with mutations in the human prohormone convertase 1 gene. Nat Genet 1997; 16: 303-306.
21. O'Rahilly S, Gray H, Humphreys P, Krook A, Polonsky K, White A, Gibson S, Taylor K, Carr C. Brief report: impaired processing of prohormones associated with abnormalities of glucose homeostasis and adrenal function. N Engl J Med 1995; 333: 1386-1390.
22. Gliemann J, Sorenson, HH. Assay of insulin-like activity by the isolated fat cell method: IV. The biological activity of proinsulin. Diabetologia 1970; 6: 499-504.
23. Robbins DC, Tager HS, Rubenstein AH. Biologic and clinical importance of proinsulin. N Engl J Med 1984; 310: 1165-1175.
24. Shoelson S, Polonsky K, Zeidler A, Rubenstein A, Tager H. Human insulin B24 (Phe→Ser). Secretion and metabolic clearance of the abnormal insulin in man and in a dog model. J Clin Invest 1984; 73: 1351-1358.
25. Rhodes CJ, Alarcón C. What β-cell defect could lead to hyperproinsulinemia in NIDDM? Some clues from recent advances made in understanding the proinsulin-processing mechanism. Diabetes 1994; 43: 511-517.
26. Sizonenko S, Irminger J-C, Buhler L, Deng S, Morel P, Halban PA. Kinetics of proinsulin conversion in human islets. Diabetes 1993; 42: 933-936.
27. Furuta M, Carroll R, Martin S, Swift H, Ravazzola M, Orci L, Steiner D. Incomplete processing of proinsulin to insulin accompanied by elevation of des-31,32-proinsulin intermediate in islets of mice lacking active PC2. J Biol Chem 1998; 273: 3431-3437.
28. Sizonenko SV, Halban PA. Differential rates of conversion of rat proinsulins I and II. Evidence for slow cleavage at the B-chain/C-peptide junction of proinsulin II. Biochem J 1991; 278: 621-625.
29. Martin SK, Carroll R, Benig M, Steiner DF. Regulation by glucose of the biosynthesis of PC2, PC3 and proinsulin in (ob/ob) mouse islets of Langerhans. FEBS Lett 1994; 356: 279-282.
30. Skelly R, Schuppin G, Ishihara H, Oka Y, Rhodes C. Glucose-regulated translational control of proinsulin biosynthesis with that of the proinsulin endopeptidases PC2 and PC3 in the insulin-producing MIN6 cell line. Diabetes 1996; 45: 37-43.
31. Ohagi S, Sakaguchi H, Sanke T, Tatsuta H, Hanabusa T, Nanjo K. Human prohormone convertase 3 gene: exon-intron organization and molecular scanning for mutations in Japanese subjects with NIDDM. Diabetes 1996; 45: 897-901.
32. Kalidas K, Dow E, Saker P, Wareham N, Halsall D, Jackson R, Chan S, Gelding S, Walker M, Kousta E, Johnston D, O'Rahilly S, McCarthy M. Prohormone convertase 1 in obesity, gestational diabetes mellitus, and NIDDM: no evidence for a major susceptibility role. Diabetes 1998; 47: 287-289.
33. Yoshida H, Ohagi S, Sanke T, Furuta H, Furuta M, Nanjo K. Association of the prohormone convertase 2 gene (PCSK2) on chromosome 20 with NIDDM in Japanese subjects. Diabetes 1995; 44: 389-393.
34. Utsunomiya N, Ohagi S, Sanke T, Tatsuta H, Hanabusa T, Nanjo K. Organization of the human carboxypeptidase E gene and molecular scanning for mutations in Japanese subjects with NIDDM or obesity. Diabetologia 1998; 41: 701-705.
35. Hostens K, Ling Z, Van Schravendijk C, Pipeleers D. Prolonged exposure of human β-cells to high glucose increases their release of proinsulin during acute stimulation with glucose or arginine. J Clin Endocrinol Metab 1999; 84: 1386-1390.
36. Seaquest E, Kahn S, Clark P, Hales C, Porte D Jr, Robertson R. Hyperproinsulinemia is associated with increased beta cell demand after hemipancreatectomy in humans. J Clin Invest 1996; 97: 455-460.
37. Furukawa H, Carroll RJ, Swift HH, Steiner DF. Long-term elevation of free fatty acids leads to delayed processing of proinsulin and prohormone convertases 2 and 3 in the pancreatic β-cell line MIN6. Diabetes 1999; 48: 1395-1401.
38. Steiner D, Tager H, Nanjo K, Chan S, Rubenstein A. Familial syndromes of hyperproinsulinemia and hyperinsulinemia with mild diabetes. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The Metabolic Basis of Inherited Disease, Vol. 1. New York: McGraw-Hill, 1995; 897-904.
39. Collinet M, Berthelon M, Benit P, Laborde K, Desbuquois B, Munnich A, Robert J. Familial hyperproinsulinaemia due to a mutation substituting histidine for arginine at position 65 in proinsulin: identification of the mutation by restriction enzyme mapping. Eur J Pediatr 1998; 157: 456-460.
40. Carroll RJ, Hammer RE, Chan SJ, Swift HH, Rubenstein AH, Steiner DF. A mutant human proinsulin is secreted from islets of Langerhans in increased amounts via an unregulated pathway. Proc Natl Acad Sci USA 1988; 85: 8943-8947.
41. B10] insulin in AtT20 cells. Mol Endocrinol 1991; 5: 319-326.
42. Gruppuso PA, Gorden P, Kahn CR, Cornblath M, Zeller P, Schwartz R. Familial hyperproinsulinemia due to a proposed defect in conversion of proinsulin to insulin. N Engl J Med 1984; 311: 629-634.
43. Oyer P, Cho S, Peterson J, Steiner D. Studies on human proinsulin: isolation and amino acid sequence of the human pancreatic C-peptide. J Biol Chem 1971; 246: 1365-1374.
44. Gorden P, Skarulis MC, Roach P, Comi RJ, Fraker DL, Norton JA, Alexander HR, Doppman JL. Plasma proinsulin-like component in insulinoma: a 25-year experience. J Clin Endocrinol Metab 1995; 80: 2884-2887.
45. Nakamori S, Ishikawa O, Ohigashi H, Sasakuma F, Shimizu T, Nakaizumi A, Furukawa H, Sasaki Y, Imaoka S. Increased blood proinsulin and decreased C-peptide levels in patients with pancreatic cancer. Hepato-Gastroenterology 1999; 46: 16-24.
46. Azzoni C, D'Adda T, Tamburrano G, Coscelli C, Madsen OD, Scopsi L, Bordi C. Functioning human insulinomas. An immunohistochemical analysis of intracellular insulin processing. Virchows Arch 1998; 433: 495-504.
47. Roth J, Komminoth P, Heitz P. Topographic abnormalities of proinsulin to insulin conversion in functioning human insulinomas. Comparison of immunoelectron microscopic and clinical data. Am J Pathol 1995; 147: 489-502.
48. +-ATPaSe (V-ATPase) in the tumor tissue. Int J Pancreatol 1999; 25: 11-16.
49. Poncin J, Abs R, Velkeniers B, Bonduelle M, Abramowicz M, Legros J-J, Verloes A, Meurisse M, Van Gaal L, Verellen C, Koulischer L, Beckers A. Mutation analysis of the MEN1 gene in Belgian patients with multiple endocrine neoplasia type 1 and related diseases. Hum Mutat 1999; 13: 54-60.
50. de Lonlay-Debeney P, Poggi-Travert F, Fournet J-C, Sempoux C, Vici CD, Brunelle F, Gouati G, Rahier J, Junien C, Nihoul-Fékété C, Robert J-J, Saudubray J-M. Clinical features of 52 neonates with hyperinsulinism. N Engl J Med 1999; 340: 1169-1175.
51. KATP: An inward rectifier subunit plus the sulfonylurea receptor. Science 1995; 270: 1166-1170.
52. Glaser B, Kesavan P, Heyman M, Davis E, Cuesta A, Buchs A, Stanley CA, Thornton PS, Permutt MA, Matschinsky FM, Herold KC. Brief report: familial hyperinsulinism caused by an activating glucokinase mutation. N Engl J Med 1998; 338: 226-230.
53. Stanley CA, Lieu YK, Hsu BY, Burlina AB, Greenbert CR, Hopwood NJ, Perlman K, Rich BH, Zammarchi E, Poncz M. Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene. N Engl J Med 1998; 338: 1352-1357.
54. Glaser B, Thornton PS, Herold K, Stanley CA. Clinical and molecular heterogeneity of familial hyperinsulinism. J Pediatr 1998; 133: 801-802.
55. Leibowitz G, Weintrob N, Pikarsky A, Josefsberg Z, Landau H, Glaser B, Hales C, Cerasi E. Normal proinsulin processing despite beta-cell dysfunction in persistent hyperinsulinaemic hypoglycaemia of infancy (nesidioblastosis). Diabetologia 1996; 39: 1338-1344.
56. Faehling M, Fussgaenger R, Jehle P. High affinity binding sites for proinsulin on human umbilical vein endothelial cells (HUVEC). Diabetologia 1999; 42: 259-260.
57. Jehle P, Lutz M, Fussgaenger R. High affinity binding sites for proinsulin in human IM-9 lymphoblasts. Diabetologia 1996; 39: 421-432.
58. Schneider DJ, Nordt TK, Sobel BE. Stimulation by proinsulin of expression of plasminogen activator inhibitor type-1 in endothelial cells. Diabetes 1992; 41: 890-895.
59. Forst T, Kunt T, Pohlmann T, Goitom K, Engelbach M, Beyer J, Pfutzner A. Biological activity of C-peptide on the skin microcirculation in patients with insulin-dependent diabetes mellitus. J Clin Invest 1998; 101: 2036-2041.
60. Dodson G, Steiner D. The role of assembly in insulin's biosynthesis. Curr Opin Struct Biol 1998; 8: 189-194.
61. Westphal C, Muller L, Zhou A, Zhu X, Bonner-Weir S, Steiner D, Lindberg I, Leder P. The neuroendocrine protein 7B2 is required for peptide hormone processing in vivo and provides a novel mechanism for pituitary Cushing's disease. Cell 1999; 96: 689-700.
62. Naggert JK, Fricker LD, Varlamov O, Nishina PM, Rouillé Y, Steiner DF, Carroll RJ, Paigen BJ, Leiter EH. Hyperproinsulinaemia in obese fat/fat mice associated with a carboxypeptidase E mutation which reduces enzyme activity. Nat Genet 1995; 10: 135-142.