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MeSH Review

Multiple Endocrine Neoplasia Type 1

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Disease relevance of Multiple Endocrine Neoplasia Type 1


High impact information on Multiple Endocrine Neoplasia Type 1

  • Multiple endocrine neoplasia-type 1 (MEN1) is an autosomal dominant familial cancer syndrome characterized by tumors in parathyroids, enteropancreatic endocrine tissues, and the anterior pituitary [6].
  • A family with isolated hyperparathyroidism segregating a missense MEN1 mutation and showing loss of the wild-type alleles in the parathyroid tumors [7].
  • Mutations of the MEN1 gene, encoding the tumor suppressor menin, predispose individuals to the cancer syndrome multiple endocrine neoplasia type 1, characterized by the development of tumors of the endocrine pancreas and anterior pituitary and parathyroid glands [8].
  • The observation that atropine inhibits serum PP levels in MEN I patients with endocrine pancreatic tumors throws doubt on the clinical usefulness of an atropine suppression test for PP in the diagnosis of pancreatic endocrine tumors [9].
  • In 5 other patients with evidence of gastrinoma from MEN I families serum PP levels were normal [9].

Chemical compound and disease context of Multiple Endocrine Neoplasia Type 1

  • Intravenous administration of 1 mg atropine to all 19 affected members of MEN I families and to 8 normal control subjects inhibited serum PP in all of them [9].
  • Reproductive hormones in aging men. I. Measurement of sex steroids, basal luteinizing hormone, and Leydig cell response to human chorionic gonadotropin [10].
  • Comparison of K. oxytoca beta-lactamases with plasmid-mediated extended-spectrum beta-lactamases MEN-1 and TOHO-1 implied that the threonine at position 168 present in OXY-2 beta-lactamase instead of the alanine in OXY-1 could be responsible for its modified substrate hydrolysis [11].
  • Plasma TG change over 8 h was determined following a liquid fatty meal in 14 male habitual runners (R) and 13 physically inactive men (I) after 3 weeks of alcohol abstinence and 3 weeks of drinking approximately 41 g (1.44 oz) of ethanol per day [12].
  • H2-antagonist therapy should be the initial treatment of choice in patients with MEN I who have ZES [13].

Biological context of Multiple Endocrine Neoplasia Type 1


Anatomical context of Multiple Endocrine Neoplasia Type 1

  • No patient with ZES and MEN I was cured of ZES despite the fact that islet cell tumor was removed from the region of the gastrin gradient in five of six patients [19].
  • A brother was found to suffer from peptic ulcer disease caused by hyperparathyroidism and, during screening for other organ involvement associated with the MEN I syndrome, two tumors were found, one (4 cm) in the pancreatic tail region and one in the right adrenal gland [20].

Gene context of Multiple Endocrine Neoplasia Type 1

  • Both beta-lactamases are distantly related to other plasmidic class A enzymes (homology to TEM-1 is 38.1% for CTX-M-1/MEN-1 and 36.5% for CTX-M-2); the closest relationship was with the chromosomal beta-lactamase of Klebsiella oxytoca E23004 (homologies of 74.5% for CTX-M-1/MEN-1 and 77.9% for CTX-M-2) [21].
  • Studies with the markers D11S480, PYGM, D11S449, and D11S987 in 13 patients (12 of whom were from our group of 16) revealed 4 losses of heterozygosity on D11 S480 on 11q13, but the deletion did not affect the MEN I gene in any case [22].
  • The prevalence of GHRH in MEN-I tumours remains to be established [23].
  • Thus, mutations in the coding segments of the HNP36 gene are not the cause of the MEN I syndrome [24].
  • We conclude that, in comparison with pituitary adenomas occurring in the general population, those occurring in association with MEN-I are (1) more often endocrinologically functional, (2) more frequently GH- or PRL-producing, and (3) clinicopathologically similar in terms of the subjects age and sex as well as of tumor size and invasiveness [25].

Analytical, diagnostic and therapeutic context of Multiple Endocrine Neoplasia Type 1


  1. Analysis of a carbapenem-hydrolyzing class A beta-lactamase from Enterobacter cloacae and of its LysR-type regulatory protein. Naas, T., Nordmann, P. Proc. Natl. Acad. Sci. U.S.A. (1994) [Pubmed]
  2. Multiple endocrine neoplasia, type I. Association with marfanoid habitus, optic atrophy, and other abnormalities. Manning, G.S., Stevens, K.A., Stock, J.L. Arch. Intern. Med. (1983) [Pubmed]
  3. Prospective study of the standard meal provocative test in Zollinger-Ellison syndrome. Frucht, H., Howard, J.M., Stark, H.A., McCarthy, D.M., Maton, P.N., Gardner, J.D., Jensen, R.T. Am. J. Med. (1989) [Pubmed]
  4. Zollinger-Ellison syndrome can be the initial endocrine manifestation in patients with multiple endocrine neoplasia-type I. Benya, R.V., Metz, D.C., Venzon, D.J., Fishbeyn, V.A., Strader, D.B., Orbuch, M., Jensen, R.T. Am. J. Med. (1994) [Pubmed]
  5. Two patients with Cushing's disease in a kindred with multiple endocrine neoplasia type I. Gaitan, D., Loosen, P.T., Orth, D.N. J. Clin. Endocrinol. Metab. (1993) [Pubmed]
  6. Positional cloning of the gene for multiple endocrine neoplasia-type 1. Chandrasekharappa, S.C., Guru, S.C., Manickam, P., Olufemi, S.E., Collins, F.S., Emmert-Buck, M.R., Debelenko, L.V., Zhuang, Z., Lubensky, I.A., Liotta, L.A., Crabtree, J.S., Wang, Y., Roe, B.A., Weisemann, J., Boguski, M.S., Agarwal, S.K., Kester, M.B., Kim, Y.S., Heppner, C., Dong, Q., Spiegel, A.M., Burns, A.L., Marx, S.J. Science (1997) [Pubmed]
  7. A family with isolated hyperparathyroidism segregating a missense MEN1 mutation and showing loss of the wild-type alleles in the parathyroid tumors. Teh, B.T., Esapa, C.T., Houlston, R., Grandell, U., Farnebo, F., Nordenskjöld, M., Pearce, C.J., Carmichael, D., Larsson, C., Harris, P.E. Am. J. Hum. Genet. (1998) [Pubmed]
  8. Conditional inactivation of the MEN1 gene leads to pancreatic and pituitary tumorigenesis but does not affect normal development of these tissues. Biondi, C.A., Gartside, M.G., Waring, P., Loffler, K.A., Stark, M.S., Magnuson, M.A., Kay, G.F., Hayward, N.K. Mol. Cell. Biol. (2004) [Pubmed]
  9. Basal and postatropine serum pancreatic polypeptide concentrations in familial multiple endocrine neoplasia type I. Lamers, C.B., Diemel, C.M. J. Clin. Endocrinol. Metab. (1982) [Pubmed]
  10. Reproductive hormones in aging men. I. Measurement of sex steroids, basal luteinizing hormone, and Leydig cell response to human chorionic gonadotropin. Harman, S.M., Tsitouras, P.D. J. Clin. Endocrinol. Metab. (1980) [Pubmed]
  11. Variability of chromosomally encoded beta-lactamases from Klebsiella oxytoca. Fournier, B., Roy, P.H. Antimicrob. Agents Chemother. (1997) [Pubmed]
  12. Effect of alcohol and exercise on postprandial lipemia and triglyceride clearance in men. Hartung, G.H., Lawrence, S.J., Reeves, R.S., Foreyt, J.P. Atherosclerosis (1993) [Pubmed]
  13. Management of the Zollinger-Ellison syndrome in patients with multiple endocrine neoplasia type I. van Heerden, J.A., Smith, S.L., Miller, L.J. Surgery (1986) [Pubmed]
  14. Molecular pathogenesis of primary hyperparathyroidism. Arnold, A., Shattuck, T.M., Mallya, S.M., Krebs, L.J., Costa, J., Gallagher, J., Wild, Y., Saucier, K. J. Bone Miner. Res. (2002) [Pubmed]
  15. Cloning and sequence of the gene encoding a cefotaxime-hydrolyzing class A beta-lactamase isolated from Escherichia coli. Ishii, Y., Ohno, A., Taguchi, H., Imajo, S., Ishiguro, M., Matsuzawa, H. Antimicrob. Agents Chemother. (1995) [Pubmed]
  16. Dexamethasone in resting and exercising men. I. Effects on bioenergetics, minerals, and related hormones. Marquet, P., Lac, G., Chassain, A.P., Habrioux, G., Galen, F.X. J. Appl. Physiol. (1999) [Pubmed]
  17. Genetics of primary aldosteronism. Gordon, R.D., Klemm, S.A., Tunny, T.J., Stowasser, M. Clin. Exp. Pharmacol. Physiol. (1994) [Pubmed]
  18. Predictive genetic screening and clinical findings in multiple endocrine neoplasia type I families. Kopp, I., Bartsch, D., Wild, A., Schilling, T., Nies, C., Bergenfelz, A., Rieder, H., Simon, B., Rothmund, M. World journal of surgery. (2001) [Pubmed]
  19. Management of islet cell tumors in patients with multiple endocrine neoplasia: a prospective study. Sheppard, B.C., Norton, J.A., Doppman, J.L., Maton, P.N., Gardner, J.D., Jensen, R.T. Surgery (1989) [Pubmed]
  20. Acromegaly caused by growth hormone-relating hormone in a patient with multiple endocrine neoplasia type I. Liu, S.W., van de Velde, C.J., Heslinga, J.M., Kievit, J., Roelfsema, F. Jpn. J. Clin. Oncol. (1996) [Pubmed]
  21. Sequences of beta-lactamase genes encoding CTX-M-1 (MEN-1) and CTX-M-2 and relationship of their amino acid sequences with those of other beta-lactamases. Bauernfeind, A., Stemplinger, I., Jungwirth, R., Ernst, S., Casellas, J.M. Antimicrob. Agents Chemother. (1996) [Pubmed]
  22. MEN I gene mutations in sporadic adrenal adenomas. Schulte, K.M., Heinze, M., Mengel, M., Simon, D., Scheuring, S., Köhrer, K., Röher, H.D. Hum. Genet. (1999) [Pubmed]
  23. Pancreatic endocrine tumour producing growth hormone-releasing hormone associated with multiple endocrine neoplasia type I syndrome. Asa, S.L., Singer, W., Kovacs, K., Horvath, E., Murray, D., Colapinto, N., Thorner, M.O. Acta Endocrinol. (1987) [Pubmed]
  24. The human HNP36 gene is localized to chromosome 11q13 and produces alternative transcripts that are not mutated in multiple endocrine neoplasia, type 1 (MEN I) syndrome. Williams, J.B., Rexer, B., Sirripurapu, S., John, S., Goldstein, R., Phillips, J.A., Haley, L.L., Sait, S.N., Shows, T.B., Smith, C.M., Gerhard, D.S. Genomics (1997) [Pubmed]
  25. Pituitary adenomas of the multiple endocrine neoplasia type I syndrome. Scheithauer, B.W., Laws, E.R., Kovacs, K., Horvath, E., Randall, R.V., Carney, J.A. Seminars in diagnostic pathology. (1987) [Pubmed]
  26. Multiple endocrine syndrome type I. Clinical, laboratory findings, and management in five families. Samaan, N.A., Ouais, S., Ordonez, N.G., Choksi, U.A., Sellin, R.V., Hickey, R.C. Cancer (1989) [Pubmed]
  27. Thymic carcinoid in association with MEN syndromes. Zeiger, M.A., Swartz, S.E., MacGillivray, D.C., Linnoila, I., Shakir, M. The American surgeon. (1992) [Pubmed]
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