multiple endocrine neoplasia type 1

Summary

Summary: A form of multiple endocrine neoplasia that is characterized by the combined occurrence of tumors in the PARATHYROID GLANDS, the PITUITARY GLAND, and the PANCREATIC ISLETS. The resulting clinical signs include HYPERPARATHYROIDISM; HYPERCALCEMIA; HYPERPROLACTINEMIA; CUSHING DISEASE; GASTRINOMA; and ZOLLINGER-ELLISON SYNDROME. This disease is due to loss-of-function of the MEN1 gene, a tumor suppressor gene (GENES, TUMOR SUPPRESSOR) on CHROMOSOME 11 (Locus: 11q13).

Top Publications

  1. ncbi Guidelines for diagnosis and therapy of MEN type 1 and type 2
    M L Brandi
    Department of Internal Medicine, University of Florence, 50139 Florence, Italy
    J Clin Endocrinol Metab 86:5658-71. 2001
  2. doi Early-onset, progressive, frequent, extensive, and severe bone mineral and renal complications in multiple endocrine neoplasia type 1-associated primary hyperparathyroidism
    Delmar M Lourenço
    Endocrine Genetics Unit LIM 25, Division of Endocrinology, University of Sao Paulo School of Medicine, Sao Paulo, Brazil
    J Bone Miner Res 25:2382-91. 2010
  3. ncbi Positional cloning of the gene for multiple endocrine neoplasia-type 1
    S C Chandrasekharappa
    Laboratory of Gene Transfer, National Human Genome Research Institute NHGRI, National Institutes of Health NIH, Bethesda, MD 20892, USA
    Science 276:404-7. 1997
  4. ncbi Is total parathyroidectomy the treatment of choice for hyperparathyroidism in multiple endocrine neoplasia type 1?
    Francesco Tonelli
    Department of Clinical Physiopathology, University of Florence, Medical SchoolFlorence, Italy
    Ann Surg 246:1075-82. 2007
  5. ncbi Multiple endocrine neoplasia type 1 (MEN1): analysis of 1336 mutations reported in the first decade following identification of the gene
    Manuel C Lemos
    Academic Endocrine Unit, Nuffield Department of Clinical Medicine, University of Oxford, Oxford Centre for Diabetes, Endocrinology and Metabolism OCDEM, Churchill Hospital, Oxford, United Kingdom
    Hum Mutat 29:22-32. 2008
  6. ncbi Results of initial operation for hyperparathyroidism in patients with multiple endocrine neoplasia type 1
    Dina M Elaraj
    Surgery Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, 9000 Rockville Pike, Bethesda, MD 20892, USA
    Surgery 134:858-64; discussion 864-5. 2003
  7. doi Bone mineral density analysis in patients with primary hyperparathyroidism associated with multiple endocrine neoplasia type 1 after total parathyroidectomy
    Flavia L Coutinho
    Endocrine Genetics Unit LIM 25, Division of Endocrinology, Hospital das Clinicas, University of Sao Paulo School of Medicine, Sao Paulo, Brazil
    Clin Endocrinol (Oxf) 72:462-8. 2010
  8. pmc Glucose-dependent insulinotropic peptide receptor overexpression in adrenocortical hyperplasia in MEN1 syndrome without loss of heterozygosity at the 11q13 locus
    Marcia Helena Soares Costa
    Unidade de Suprarrenal, do Hospital das Clinicas da Faculdade de Medicina da Universidade de São Paulo, SP, Brasil
    Clinics (Sao Paulo) 66:529-33. 2011
  9. ncbi Pituitary disease in MEN type 1 (MEN1): data from the France-Belgium MEN1 multicenter study
    Bruno Verges
    Department of Endocrinology, University Hospital, 21000 Dijon, France
    J Clin Endocrinol Metab 87:457-65. 2002
  10. doi The MEN1 gene and pituitary tumours
    Sunita K Agarwal
    Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA
    Horm Res 71:131-8. 2009

Detail Information

Publications316 found, 100 shown here

  1. ncbi Guidelines for diagnosis and therapy of MEN type 1 and type 2
    M L Brandi
    Department of Internal Medicine, University of Florence, 50139 Florence, Italy
    J Clin Endocrinol Metab 86:5658-71. 2001
    ....
  2. doi Early-onset, progressive, frequent, extensive, and severe bone mineral and renal complications in multiple endocrine neoplasia type 1-associated primary hyperparathyroidism
    Delmar M Lourenço
    Endocrine Genetics Unit LIM 25, Division of Endocrinology, University of Sao Paulo School of Medicine, Sao Paulo, Brazil
    J Bone Miner Res 25:2382-91. 2010
    Differences in bone mineral density (BMD) patterns have been recently reported between multiple endocrine neoplasia type 1-related primary hyperparathyroidism (HPT/MEN1) and sporadic primary HPT...
  3. ncbi Positional cloning of the gene for multiple endocrine neoplasia-type 1
    S C Chandrasekharappa
    Laboratory of Gene Transfer, National Human Genome Research Institute NHGRI, National Institutes of Health NIH, Bethesda, MD 20892, USA
    Science 276:404-7. 1997
    ..The identification of MEN1 will enable improved understanding of the mechanism of endocrine tumorigenesis and should facilitate early diagnosis...
  4. ncbi Is total parathyroidectomy the treatment of choice for hyperparathyroidism in multiple endocrine neoplasia type 1?
    Francesco Tonelli
    Department of Clinical Physiopathology, University of Florence, Medical SchoolFlorence, Italy
    Ann Surg 246:1075-82. 2007
    ..The aim of the present report is to describe the results obtained with total parathyroidectomy (TPTX) guided by rapid intraoperative parathyroid hormone (PTH) evaluation, followed by immediate parathyroid autograft with fresh tissue...
  5. ncbi Multiple endocrine neoplasia type 1 (MEN1): analysis of 1336 mutations reported in the first decade following identification of the gene
    Manuel C Lemos
    Academic Endocrine Unit, Nuffield Department of Clinical Medicine, University of Oxford, Oxford Centre for Diabetes, Endocrinology and Metabolism OCDEM, Churchill Hospital, Oxford, United Kingdom
    Hum Mutat 29:22-32. 2008
    b>Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by the occurrence of tumors of the parathyroids, pancreas, and anterior pituitary...
  6. ncbi Results of initial operation for hyperparathyroidism in patients with multiple endocrine neoplasia type 1
    Dina M Elaraj
    Surgery Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, 9000 Rockville Pike, Bethesda, MD 20892, USA
    Surgery 134:858-64; discussion 864-5. 2003
    Hyperparathyroidism in patients with multiple endocrine neoplasia type 1 (MEN1) is characterized by multiglandular disease and a propensity for recurrence after parathyroidectomy (PTx)...
  7. doi Bone mineral density analysis in patients with primary hyperparathyroidism associated with multiple endocrine neoplasia type 1 after total parathyroidectomy
    Flavia L Coutinho
    Endocrine Genetics Unit LIM 25, Division of Endocrinology, Hospital das Clinicas, University of Sao Paulo School of Medicine, Sao Paulo, Brazil
    Clin Endocrinol (Oxf) 72:462-8. 2010
    ..PTx) on bone mineral density (BMD) in the setting of patients with hyperparathyroidism (HPT) associated with multiple endocrine neoplasia type 1 (MEN1). This study investigates the impact of total PTx on BMD in patients with HPT/MEN1.
  8. pmc Glucose-dependent insulinotropic peptide receptor overexpression in adrenocortical hyperplasia in MEN1 syndrome without loss of heterozygosity at the 11q13 locus
    Marcia Helena Soares Costa
    Unidade de Suprarrenal, do Hospital das Clinicas da Faculdade de Medicina da Universidade de São Paulo, SP, Brasil
    Clinics (Sao Paulo) 66:529-33. 2011
    ..However, to our knowledge, the status of glucose-dependent insulinotropic peptide receptor expression in adrenocortical lesions in MEN1 has not been previously investigated...
  9. ncbi Pituitary disease in MEN type 1 (MEN1): data from the France-Belgium MEN1 multicenter study
    Bruno Verges
    Department of Endocrinology, University Hospital, 21000 Dijon, France
    J Clin Endocrinol Metab 87:457-65. 2002
    ..Our study, based on a large group of MEN1 patients, shows that pituitary adenomas occur in 42% of the cases and are characterized by a larger size and a more aggressive presentation than without MEN1...
  10. doi The MEN1 gene and pituitary tumours
    Sunita K Agarwal
    Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA
    Horm Res 71:131-8. 2009
    Sporadic multiple endocrine neoplasia type 1 (MEN1) is defined as the occurrence of tumours in two of three main endocrine tissue types: parathyroid, pituitary and pancreaticoduodenal...
  11. ncbi Parathyroid surgery in familial hyperparathyroid disorders
    T Carling
    Department of Surgery, Yale University School of Medicine, New Haven, CT, USA
    J Intern Med 257:27-37. 2005
    ..b>Multiple endocrine neoplasia type 1 (MEN1) is treated with either subtotal parathyroidectomy or total parathyroidectomy with immediate ..
  12. pmc Epidemiology data on 108 MEN 1 patients from the GTE with isolated nonfunctioning tumors of the pancreas
    Frederic Triponez
    General and Endocrine Surgery, University Hospital, Lille, France
    Ann Surg 243:265-72. 2006
    ..To analyze the penetrance and clinical course of isolated nonfunctioning tumors of the pancreas (NFTP) in MEN 1 patients, and to propose a strategy for managing them...
  13. doi Multiple endocrine neoplasia type 1 (MEN1): not only inherited endocrine tumors
    Alberto Falchetti
    Regional Centre for Hereditary Endocrine Tumors, Unit of Metabolic Bone Diseases, Department of Internal Medicine, University of Florence, Florence, Italy
    Genet Med 11:825-35. 2009
    ..The ever increrasing combination of genetic and clinical tools will allow early detection of MEN1-associated neoplasms, potentially improving clinical outcomes and quality of life for both affected patients and their relatives...
  14. ncbi Identification of MEN1 gene mutations in sporadic carcinoid tumors of the lung
    L V Debelenko
    Laboratory of Pathology, National Cancer Institute, NIH, Bethesda, MD 20892, USA
    Hum Mol Genet 6:2285-90. 1997
    Lung carcinoids occur sporadically and rarely in association with multiple endocrine neoplasia type 1 (MEN1). There are no well defined genetic abnormalities known to occur in these tumors...
  15. ncbi Common ancestral mutation in the MEN1 gene is likely responsible for the prolactinoma variant of MEN1 (MEN1Burin) in four kindreds from Newfoundland
    S E Olufemi
    Laboratory of Gene Transfer, National Human Genome Research Institute, NIH, Bethesda, Maryland 20892 4442, USA
    Hum Mutat 11:264-9. 1998
    Familial multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder with affected individuals developing parathyroid, gastrointestinal (GI) endocrine, and anterior pituitary tumors...
  16. doi Gender-related differences in MEN1 lesion occurrence and diagnosis: a cohort study of 734 cases from the Groupe d'etude des Tumeurs Endocrines
    P Goudet
    Centre Hospitalier Universitaire de Dijon, Service de Chirurgie Endocrinienne, Dijon, France
    Eur J Endocrinol 165:97-105. 2011
    b>Multiple endocrine neoplasia type 1 (MEN1) disease is an autosomal dominant syndrome that is believed to equally affect men and women. This assumption has never been confirmed.
  17. pmc Multiple endocrine neoplasia type 1 knockout mice develop parathyroid, pancreatic, pituitary and adrenal tumours with hypercalcaemia, hypophosphataemia and hypercorticosteronaemia
    Brian Harding
    Academic Endocrine Unit, Nuffield Department of Clinical Medicine, Oxford Centre for Diabetes, Endocrinology and Metabolism OCDEM, Churchill Hospital, University of Oxford, Oxford, UK
    Endocr Relat Cancer 16:1313-27. 2009
    b>Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized in man by parathyroid, pancreatic, pituitary and adrenal tumours...
  18. ncbi Subtotal parathyroidectomy as an adequate treatment for primary hyperparathyroidism in multiple endocrine neoplasia type 1
    Johnathan G H Hubbard
    Department of Endocrine Surgery, University Hospital La Timone, Marseille, France
    Arch Surg 141:235-9. 2006
    The most appropriate surgical approach for hyperparathyroidism (HPT) in multiple endocrine neoplasia type 1 remains controversial...
  19. doi Multiple endocrine neoplasia type 1: a chromatin writer's block
    K M A Dreijerink
    Department of Physiological Chemistry, University Medical Center Utrecht, Utrecht 3508 AB, The Netherlands
    J Intern Med 266:53-9. 2009
    b>Multiple endocrine neoplasia type 1 (MEN1) is caused by inactivating germ line mutations of the MEN1 tumour suppressor gene...
  20. ncbi Adrenal involvement in multiple endocrine neoplasia type 1: results of 7 years prospective screening
    J Waldmann
    Department of General Surgery, Philipps University, Marburg, Germany
    Langenbecks Arch Surg 392:437-43. 2007
    Adrenal tumors are a common manifestation of the multiple endocrine neoplasia type 1 (MEN-1) syndrome. Prevalence in recent studies varies between 9 and 45%...
  21. ncbi Adrenal involvement in multiple endocrine neoplasia type 1
    Peter Langer
    Department of Surgery, Philipps University Hospital, Baldingerstrasse, D 35043 Marburg, Germany
    World J Surg 26:891-6. 2002
    Adrenal lesions belong to the spectrum of multiple endocrine neoplasia type 1 (MEN-1) syndrome...
  22. doi A patient with MEN1-associated hyperparathyroidism, responsive to cinacalcet
    Alberto Falchetti
    University Hospital of Careggi, Florence, Italy
    Nat Clin Pract Endocrinol Metab 4:351-7. 2008
    A 30-year-old woman with suspected multiple endocrine neoplasia type 1 (MEN1) was referred to our center in 2001 with primary hyperparathyroidism caused by a multiglandular parathyroid adenoma...
  23. pmc Isolated familial somatotropinoma: 11q13-loh and gene/protein expression analysis suggests a possible involvement of aip also in non-pituitary tumorigenesis
    Rodrigo A Toledo
    Unidade de Endocrinologia Genética, LIM 25, Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil
    Clinics (Sao Paulo) 65:407-15. 2010
    ..However, no detailed investigations of non-pituitary tumors of AIP-mutated patients have been reported so far...
  24. ncbi Familial isolated hyperparathyroidism: clinical and genetic characteristics of 36 kindreds
    William F Simonds
    Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland 20892 1752, USA
    Medicine (Baltimore) 81:1-26. 2002
  25. ncbi Molecular genetics of multiple endocrine neoplasia types 1 and 2
    Stephen J Marx
    National Institutes of Health, Building 10, Room 9C 101, Bethesda, Maryland 20892 1802, USA
    Nat Rev Cancer 5:367-75. 2005
    ..The recent discovery of the main gene in each MEN syndrome has furthered our understanding of not only hereditary but also sporadic tumours and has fostered new avenues of research...
  26. ncbi Menin and its interacting proteins: elucidation of menin function
    Katalin Balogh
    Second Department of Medicine, Semmelweis University, Szentkiralyi str 46, H 1088 Budapest, Hungary
    Trends Endocrinol Metab 17:357-64. 2006
    The multiple endocrine neoplasia type 1 (MEN1) gene is a tumor suppressor gene encoding a 610 amino acid nuclear protein, menin...
  27. pmc Characterization of mutations in patients with multiple endocrine neoplasia type 1
    J H Bassett
    MRC Molecular Endocrinology Group, MRC Clinical Sciences Centre, Imperial School of Medicine, Hammersmith Hospital, London W12 0NN, United Kingdom
    Am J Hum Genet 62:232-44. 1998
    b>Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by tumors of the parathyroids, pancreatic islets, and anterior pituitary...
  28. ncbi Familial isolated primary hyperparathyroidism--a multiple endocrine neoplasia type 1 variant?
    S Miedlich
    Medical Department III, University of Leipzig, Germany
    Eur J Endocrinol 145:155-60. 2001
    ..Hereditary syndromes associated with primary hyperparathyroidism are multiple endocrine neoplasia type 1 and type 2 (MEN 1 and MEN 2)...
  29. ncbi Pancreatic tumours as part of the MEN-1 syndrome
    Goran Akerstrom
    Department of Surgical Sciences, University Hospital, SE 751 85 Uppsala, Sweden
    Best Pract Res Clin Gastroenterol 19:819-30. 2005
    ..This strategy with early and aggressive surgery is believed to reduce the risks for malignant progression...
  30. ncbi Clinical testing for mutations in the MEN1 gene in Sweden: a report on 200 unrelated cases
    Emma Tham
    Department of Molecular Medicine and Surgery, Karolinska Institutet, 171 76 Stockholm, Sweden
    J Clin Endocrinol Metab 92:3389-95. 2007
    b>Multiple endocrine neoplasia type 1 (MEN1) is a tumor syndrome of the parathyroid, endocrine pancreas, and anterior pituitary caused by mutations in the MEN1 gene on 11q13.
  31. doi Pituitary tumors and hyperplasia in multiple endocrine neoplasia type 1 syndrome (MEN1): a case-control study in a series of 77 patients versus 2509 non-MEN1 patients
    Jacqueline Trouillas
    INSERM, U842, Lyon, F 69372, France
    Am J Surg Pathol 32:534-43. 2008
    ..All types of mutation were observed, including frameshifts, nonsenses, missenses, and 1 case of germline MEN1 encompassing large deletion, strongly suggesting the absence of any phenotype-genotype correlation...
  32. ncbi Management of pancreatic endocrine tumors in multiple endocrine neoplasia type 1
    Maria A Kouvaraki
    Department of Surgical Oncology, The University of Texas M D Anderson Cancer Center, Unit 444, 1400 Holcombe Boulevard, Houston, Texas 77030 1402, USA
    World J Surg 30:643-53. 2006
    Pancreatic endocrine tumors (PETs) occur in at least 50% of patients with multiple endocrine neoplasia type 1 (MEN1) and are the leading cause of disease-specific mortality...
  33. ncbi Gastric carcinoid tumors in multiple endocrine neoplasia-1 patients with Zollinger-Ellison syndrome can be symptomatic, demonstrate aggressive growth, and require surgical treatment
    Jeffrey A Norton
    Department of Surgery, Stanford University Medical Center, 300 Pasteur Drive, Stanford, CA 94305 5651, USA
    Surgery 136:1267-74. 2004
    ..We report 5 patients with MEN-1/ZES with symptomatic, aggressive gastric carcinoid tumors that required surgical procedures...
  34. doi Care for patients with multiple endocrine neoplasia type 1: the current evidence base
    C R C Pieterman
    Department of Internal Medicine, University Medical Centre Utrecht, Utrecht, The Netherlands
    Fam Cancer 10:157-71. 2011
    b>Multiple endocrine neoplasia type 1 (MEN1) is a rare disease caused by mutations in the MEN1 gene on chromosome 11...
  35. doi MEN1 missense mutations impair sensitization to apoptosis induced by wild-type menin in endocrine pancreatic tumor cells
    Wissam Bazzi
    INSERM, U865, IFR 62, Lyon, France
    Gastroenterology 135:1698-1709.e2. 2008
    Missense mutations account for 30% of mutations identified in patients with the multiple endocrine neoplasia type 1 (MEN1) syndrome...
  36. ncbi [Multiple endocrine neoplasia: a clinical model for applying molecular genetic techniques]
    N Wohllk
    Departamento de Medicina, Hospital del Salvador, Santiago de Chile
    Rev Med Chil 128:791-800. 2000
    ..Some cases of sporadic MTC are actually MEN 2A or Familial MTC after c-Ret testing is done, therefore routine application of this test is recommended in all cases of apparent sporadic MTC...
  37. ncbi Screening of the Men1 gene and discovery of germ-line and somatic mutations in apparently sporadic parathyroid tumors
    S Uchino
    Noguchi Thyroid Clinic and Hospital Foundation, Oita, Japan
    Cancer Res 60:5553-7. 2000
    ..Germ-line MEN1 gene analysis can distinguish heritable from nonheritable parathyroid tumors, and MEN1 gene evaluation of patients with apparently sporadic parathyroid tumor is recommended before parathyroid surgery...
  38. ncbi Multiple endocrine neoplasia
    R V Thakker
    Molecular Endocrinology Group, Nuffield Department of Clinical Medicine, University of Oxford, John Radcliffe Hospital, Headington, Oxford OX3 9DU, UK
    Horm Res 56:67-72. 2001
    ..Specific mutations of c-ret have been identified for each of the three MEN2 variants and mutational analysis has been used in the diagnosis and management of patients and families with the MEN2 variants...
  39. pmc Inherited pancreatic endocrine tumor syndromes: advances in molecular pathogenesis, diagnosis, management, and controversies
    Robert T Jensen
    Digestive Diseases Branch, National Institutes of Diabetes, Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA
    Cancer 113:1807-43. 2008
    Pancreatic endocrine tumors (PETs) can occur as part of 4 inherited disorders, including Multiple Endocrine Neoplasia type 1 (MEN1), von Hippel-Lindau disease (VHL), neurofibromatosis 1 (NF-1) (von Recklinghausen disease), and the ..
  40. doi Menin dynamics and functional insight: take your partners
    Katalin Balogh
    2nd Department of Medicine, Semmelweis University, 46 Szentkiralyi, Budapest H 1088, Hungary
    Mol Cell Endocrinol 326:80-4. 2010
    ..number of recent findings and novelties, menin, the protein encoded by the gene responsible for multiple endocrine neoplasia type 1 syndrome, still remains a mystery...
  41. ncbi Long-term results of less than total parathyroidectomy for hyperparathyroidism in multiple endocrine neoplasia type 1
    Laurent C Arnalsteen
    Department of General and Endocrine Surgery, University Hospital of Lille, 1 rue Michel Polonovski, 59037 Lille Cedex, France
    Surgery 132:1119-24; discussion 1124-5. 2002
    Our aim was to assess long-term results after less than total parathyroidectomy for hyperparathyroidism in multiple endocrine neoplasia type 1.
  42. ncbi Molecular pathology of parathyroid tumors
    T Carling
    Endocrine Surgery Unit, Dept Surgery, Uppsala University Hospital, Uppsala, Sweden and the Burnham Institute, La Jolla, CA, USA
    Trends Endocrinol Metab 12:53-8. 2001
    ..Several familial hyperparathyroid disorders have been studied, and the identification and characterization of the disease-causing genes have contributed to our understanding of parathyroid physiology and pathophysiology...
  43. ncbi Current concepts in the surgical management of multiple endocrine neoplasia type 1 pancreatic-duodenal disease. Results in the treatment of 40 patients with Zollinger-Ellison syndrome, hypoglycaemia or both
    N W Thompson
    Division of Endocrine Surgery, University of Michigan, Ann Arbor, USA
    J Intern Med 243:495-500. 1998
    The management of multiple endocrine neoplasia type 1 (MEN-1) pancreatic-duodenal disease, particularly when the Zollinger-Ellison syndrome (ZES) is the presenting manifestation, has remained controversial...
  44. doi Clinical and molecular genetics of acromegaly: MEN1, Carney complex, McCune-Albright syndrome, familial acromegaly and genetic defects in sporadic tumors
    Anelia Horvath
    Section on Endocrinology and Genetics, Program on Developmental Endocrinology and Genetics, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892 1103, USA
    Rev Endocr Metab Disord 9:1-11. 2008
    ..In conclusion, we attempt to present an overall, integrative approach to the human molecular genetics of both familiar and sporadic pituitary tumors...
  45. pmc Recapitulation of pancreatic neuroendocrine tumors in human multiple endocrine neoplasia type I syndrome via Pdx1-directed inactivation of Men1
    H C Jennifer Shen
    Tumor Angiogenesis Section, Surgery Branch, National Cancer Institute, Bethesda, Maryland 20892, USA
    Cancer Res 69:1858-66. 2009
    b>Multiple endocrine neoplasia type 1 (MEN1) is an autosomal syndrome caused by mutations in the MEN1 tumor suppressor gene...
  46. pmc Menin missense mutants associated with multiple endocrine neoplasia type 1 are rapidly degraded via the ubiquitin-proteasome pathway
    Hiroko Yaguchi
    Tumor Endocrinology Project, National Cancer Center Research Institute, 5 1 1 Tsukiji, Chuo Ku, Tokyo 104 0045, Japan
    Mol Cell Biol 24:6569-80. 2004
    MEN1 is a tumor suppressor gene that is responsible for multiple endocrine neoplasia type 1 (MEN1) and that encodes a 610-amino-acid protein, called menin...
  47. ncbi Natural course of small, asymptomatic neuroendocrine pancreatic tumours in multiple endocrine neoplasia type 1: an endoscopic ultrasound imaging study
    P H Kann
    Division of Endocrinology and Diabetology, Department of Surgery, Phillipp s University, Marburg, Germany
    Endocr Relat Cancer 13:1195-202. 2006
    ..and experience with EUS imaging is limited, there is no clear evidence for clinical management in multiple endocrine neoplasia type 1 (MEN1)...
  48. pmc Duodenopancreatic resections in patients with multiple endocrine neoplasia type 1
    T C Lairmore
    Section of Endocrine and Oncologic Surgery, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Ann Surg 231:909-18. 2000
    ..with a surgical approach for pancreatic and duodenal neuroendocrine tumors (NETs) in patients with multiple endocrine neoplasia type 1 (MEN 1) designed to remove all gross tumor with limited complications, preserving pancreatic ..
  49. ncbi Thymic carcinoids in multiple endocrine neoplasia type 1
    B T Teh
    Department of Molecular Medicine, Karolinska Hospital, Stockholm, Sweden
    J Intern Med 243:501-4. 1998
    Thymic carcinoid is a rare malignancy with about 150 cases reported to date. It is associated with multiple endocrine neoplasia type 1 (MEN-1), but compared with other MEN-1-related neoplasia little is known about it...
  50. ncbi Regression of type II gastric carcinoids in multiple endocrine neoplasia type 1 patients with Zollinger-Ellison syndrome after surgical excision of all gastrinomas
    Melanie L Richards
    Department of Surgery, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Drive, San Antonio, Texas 78284, USA
    World J Surg 28:652-8. 2004
    ..hypergastrinemia secondary to chronic atrophic gastritis or with Zollinger-Ellison syndrome and multiple endocrine neoplasia type 1 (ZES-MEN-1)...
  51. pmc Resolved and unresolved controversies in the surgical management of patients with Zollinger-Ellison syndrome
    Jeffrey A Norton
    Department of Surgery, Stanford University Medical Center, Stanford, California 94305 5641, USA
    Ann Surg 240:757-73. 2004
    ..Highlight unresolved controversies in the management of Zollinger-Ellison syndrome (ZES)...
  52. doi Familial pituitary tumor syndromes
    Vladimir Vasilev
    Department of Endocrinology, University of Liege, Belgium Clinical Center of Endocrinology and Gerontology, Medical University, Sofia, Bulgaria
    Endocr Pract 17:41-6. 2011
    ..To summarize current knowledge on the clinical and genetic characteristics of familial pituitary tumor syndromes...
  53. ncbi Multiple endocrine neoplasia syndrome: genetic basis for clinical management
    Tobias Carling
    Department of Surgery, Yale University School of Medicine, New Haven, CT 06510, USA
    Curr Opin Oncol 17:7-12. 2005
    ..Improved understanding of the molecular and clinical genetics associated with these lesions will likely enhance the diagnosis and treatment of patients with these diseases...
  54. ncbi Pituitary macroadenoma in a 5-year-old: an early expression of multiple endocrine neoplasia type 1
    C A Stratakis
    Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institute of Health, Bethesda, MD 20892 1862, USA
    J Clin Endocrinol Metab 85:4776-80. 2000
    b>Multiple endocrine neoplasia type 1 (MEN 1) is associated with parathyroid, enteropancreatic, pituitary, and other tumors. The MEN1 gene, a tumor suppressor, is located on chromosome 11...
  55. doi The optimal surgical treatment for primary hyperparathyroidism in MEN1 patients: a systematic review
    Jennifer M J Schreinemakers
    Department of Surgery, University Medical Center Utrecht, Heidelberglaan 100, 3584CX, Utrecht, The Netherlands
    World J Surg 35:1993-2005. 2011
    ..The optimal surgical approach for patients with primary hyperparathyroidism (pHPT) and multiple endocrine neoplasia 1 (MEN1) is controversial. We sought to determine the optimal type of surgery for pHPT in MEN1...
  56. ncbi Gene dose mapping delineated boundaries of a large germline deletion responsible for multiple endocrine neoplasia type 1
    Mariko Kikuchi
    Tumor Endocrinology Project, National Cancer Center Research Institute, 5 1 1 Tsukiji, Chuo Ku, Tokyo 104 0045, Japan
    Cancer Lett 208:81-8. 2004
    A deleted genomic region including the MEN1 gene was determined in a family with multiple endocrine neoplasia type 1 caused by a large germline deletion...
  57. pmc Parathyroid tumor development involves deregulation of homeobox genes
    H C Jennifer Shen
    Tumor Angiogenesis Section, Surgery Branch, National Cancer Institute, NIH, Bethesda, MD 20892, USA
    Endocr Relat Cancer 15:267-75. 2008
    b>Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant syndrome caused by mutations in the MEN1 tumor suppressor gene...
  58. pmc The role of germline AIP, MEN1, PRKAR1A, CDKN1B and CDKN2C mutations in causing pituitary adenomas in a large cohort of children, adolescents, and patients with genetic syndromes
    C A Stratakis
    Section on Endocrinology Genetics, Program on Developmental Endocrinology Genetics PDEGEN, Eunice Kennedy Shriver National Institute of Child Health and Human Development NICHD, National Institute of Health NIH, Bethesda, MD 20892, USA
    Clin Genet 78:457-63. 2010
    ..Thus, germline AIP or MEN1 gene mutations are frequent among pediatric patients with GH- or PRL-secreting PA but are significantly rarer in pediatric CD; PRKAR1A mutations are not present in PA outside of Carney complex...
  59. ncbi The parathyroid/pituitary variant of multiple endocrine neoplasia type 1 usually has causes other than p27Kip1 mutations
    Atsushi Ozawa
    National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892 1802, USA
    J Clin Endocrinol Metab 92:1948-51. 2007
    One variant of multiple endocrine neoplasia type 1 (MEN1) is defined by sporadic tumors of both the parathyroids and pituitary. The prevalence of identified MEN1 mutations in this variant is lower than in familial MEN1 (7% vs...
  60. doi Multiple Endocrine Neoplasia type 1
    Grzegorz Piecha
    Department of Nephrology, Endocrinology and Metabolic Diseases, Medical University of Silesia, Katowice, ul Francuska 20 24, 40 027 Katowice, Poland
    Eur J Intern Med 19:99-103. 2008
    ..parathyroid hyperplasia with pancreatic endocrine tumours and/or pituitary adenoma is classified as Multiple Endocrine Neoplasia type 1 (MEN-1) and is caused by a germ-line mutation in MEN-1 gene encoding a tumour suppressor protein, ..
  61. ncbi MEN1 mutation analysis in Chinese patients with multiple endocrine neoplasia type 1
    Xiao hua Jiang
    Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrinology and Metabolism and Chinese French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, China
    Endocr Relat Cancer 14:1073-9. 2007
    b>Multiple endocrine neoplasia type 1 (MEN1) is an inherited tumour syndrome characterized by the development of tumours of the parathyroid, anterior pituitary and pancreatic islets, etc...
  62. ncbi Analysis of gross deletions in the MEN1 gene in patients with multiple endocrine neoplasia type 1
    Martina Owens
    Department of Molecular Genetics, Royal Devon and Exeter Hospital, Peninsula Medical School, Exeter, UK
    Clin Endocrinol (Oxf) 68:350-4. 2008
    Mutation analysis with direct DNA sequencing is commonly used for the molecular diagnosis of multiple endocrine neoplasia type 1 (MEN1)...
  63. ncbi Cushing's disease as the first clinical manifestation of multiple endocrine neoplasia type 1 (MEN1) associated with an R460X mutation of the MEN1 gene
    Lisa N Matsuzaki
    Clin Endocrinol (Oxf) 60:142-3. 2004
  64. ncbi The surgical management of MEN-1 pancreatoduodenal neuroendocrine disease
    Mark S Hausman
    Division of Endocrine Surgery, Department of Surgery, University of Michigan, Taubman Center, 1500 East Medical Center Drive, Ann Arbor, MI 48109 0331, USA
    Surgery 136:1205-11. 2004
    ..An aggressive surgical approach is intended to control the functional syndromes and malignant potential for nodal or distant metastasis...
  65. ncbi Is surgery beneficial for MEN1 patients with small (< or = 2 cm), nonfunctioning pancreaticoduodenal endocrine tumor? An analysis of 65 patients from the GTE
    Frederic Triponez
    Department of General and Endocrine Surgery, University Hospital, Lille, France
    World J Surg 30:654-62; discussion 663-4. 2006
    The management of small, nonfunctioning pancreaticoduodenal endocrine tumors (NFPET) in multiple endocrine neoplasia type 1 (MEN1) patients is still controversial...
  66. ncbi Plasma chromogranin A in patients with sporadic gastro-entero-pancreatic neuroendocrine tumors or multiple endocrine neoplasia type 1
    M Peracchi
    Institute of Endocrine Sciences, University of Milan, Ospedale Maggiore IRCCS, Padiglione Granelli, Via F Sforza 35, 20122, Milan, Italy
    Eur J Endocrinol 148:39-43. 2003
    ..marker so far available, we evaluated the usefulness of CgA determination in the clinical assessment of patients with sporadic gastro-entero-pancreatic neuroendocrine tumors (GEP NETs) or multiple endocrine neoplasia type 1 (MEN 1).
  67. pmc Pancreatectomy in multiple endocrine neoplasia type 1-related gastrinomas and pancreatic endocrine neoplasias
    Francesco Tonelli
    Department of Clinical Physiopathology, University of Florence, Medical School, Florence, Italy
    Ann Surg 244:61-70. 2006
    The aim of this study was to evaluate the results of pancreatic resection in pancreatic endocrine neoplasias (PENs) in patients affected by multiple endocrine neoplasia type 1 (MEN1) syndrome.
  68. ncbi Malignant thymic carcinoid is not prevented by transcervical thymectomy in multiple endocrine neoplasia type 1
    J R Burgess
    Department of Diabetes, Royal Hobart Hospital, Tasmania, Australia
    Clin Endocrinol (Oxf) 55:689-93. 2001
    b>Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant tumour syndrome. It is characterized by primary hyperparathyroidism, pituitary neoplasia and foregut lineage neuroendocrine neoplasia...
  69. ncbi Functional interaction between tumor suppressor menin and activator of S-phase kinase
    Robert W Schnepp
    Abramson Family Cancer Research Institute, Department of Cancer Biology, University of Pennsylvania, Philadelphia, Pennsylvania 19104 6160, USA
    Cancer Res 64:6791-6. 2004
    ..Together, these findings demonstrate a functional link between menin and ASK in the regulation of cell proliferation...
  70. pmc Menin-mediated caspase 8 expression in suppressing multiple endocrine neoplasia type 1
    Ping La
    Abramson Family Cancer Research Institute, Department of Cancer Biology, Abramson Cancer Center, University of Pennsylvania, Philadelphia, Pennsylvania 19104 6160, USA
    J Biol Chem 282:31332-40. 2007
    b>Multiple endocrine neoplasia type 1 (MEN1) is a familial tumor syndrome linked to mutation of the MEN1 gene, which encodes a tumor suppressor, menin...
  71. ncbi Germline CDKN1B/p27Kip1 mutation in multiple endocrine neoplasia
    Marianthi Georgitsi
    Department of Medical Genetics, University of Helsinki, Finland
    J Clin Endocrinol Metab 92:3321-5. 2007
    Germline mutations in the MEN1 gene predispose to multiple endocrine neoplasia type 1 (MEN1) syndrome, but in up to 20-25% of clinical MEN1 cases, no MEN1 mutations can be found...
  72. ncbi Current surgical management of Zollinger-Ellison syndrome (ZES) in patients without multiple endocrine neoplasia-type 1 (MEN1)
    Jeffrey A Norton
    Department of Surgery, University of California San Francisco, 533 Parnassus Avenue, Room U 372, San Francisco, CA 94143 0790, USA
    Surg Oncol 12:145-51. 2003
    The role of surgery in the management of patients with sporadic (not part of multiple endocrine neoplasia type 1) Zollinger-Ellison syndrome (ZES) is controversial...
  73. ncbi Role of surgery in Zollinger-Ellison syndrome
    Jeffrey A Norton
    Surgical Oncology, Department of Surgery, Stanford University, Palo Alto, California, USA
    J Am Coll Surg 205:S34-7. 2007
  74. ncbi Long-term follow-up of patients with multiple endocrine neoplasia type 1
    Akihiro Sakurai
    Department of Aging Medicine and Geriatrics, Shinshu University Graduate School of Medicine, Japan
    Endocr J 54:295-302. 2007
    ..islet cell tumor (NFPT) provides a favorable quality of life and life expectancy in patients with multiple endocrine neoplasia type 1 (MEN1) remains controversial...
  75. ncbi Quality of life in patients with multiple endocrine neoplasia type 1 (MEN 1)
    G Berglund
    Department of Public Health and Caring Sciences, Uppsala University, Uppsala Science Park, S 751 83, Uppsala, Sweden
    Fam Cancer 2:27-33. 2003
    ..To study quality of life among patients living with a hereditary tumor syndrome, the small group with multiple endocrine neoplasia type 1 (MEN1) was selected...
  76. ncbi Precursor lesions in patients with multiple endocrine neoplasia type 1-associated duodenal gastrinomas
    Martin Anlauf
    Department of Pathology, Univeristy of Kiel, Germany
    Gastroenterology 128:1187-98. 2005
    ..Precursor lesions may precede the development of duodenal gastrinomas because, in contrast to sporadic gastrinomas, these tumors are usually multiple...
  77. pmc Post-surgical follow-up of primary hyperparathyroidism associated with multiple endocrine neoplasia type 1
    Flavia L Coutinho
    Endocrine Genetics Unit LIM 25, Endocrinology Division, Hospital das Clinicas, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil
    Clinics (Sao Paulo) 67:169-72. 2012
    ..investigators, but few have investigated this topic in primary hyperparathyroidism associated with multiple endocrine neoplasia type 1. Further, as far as we know, only two studies have consistently evaluated bone mineral density ..
  78. ncbi Phenotype and phenocopy: the relationship between genotype and clinical phenotype in a single large family with multiple endocrine neoplasia type 1 (MEN 1)
    J R Burgess
    Departments of Diabetes and Endocrine Services Clinical Chemistry, Royal Hobart Hospital, Hobart, Australia
    Clin Endocrinol (Oxf) 53:205-11. 2000
    The majority of reports describing the natural history and prognosis of multiple endocrine neoplasia type 1 (MEN 1) utilize phenotypic rather than molecular genetic criteria to establish a diagnosis of MEN 1.
  79. doi Asymptomatic children with multiple endocrine neoplasia type 1 mutations may harbor nonfunctioning pancreatic neuroendocrine tumors
    Paul J Newey
    Academic Endocrine Unit, Nuffield Department of Clinical Medicine, University of Oxford, Oxford Centre for Diabetes, Endocrinology, and Metabolism OCDEM, Churchill Hospital, Headington Oxford, OX3 7LJ, United Kingdom
    J Clin Endocrinol Metab 94:3640-6. 2009
    b>Multiple endocrine neoplasia type 1 (MEN1) is characterized by the occurrence of parathyroid, pituitary, and pancreatic tumors...
  80. ncbi Attenuated expression of menin and p27 (Kip1) in an aggressive case of multiple endocrine neoplasia type 1 (MEN1) associated with an atypical prolactinoma and a malignant pancreatic endocrine tumor
    Emi Ishida
    Department of Medicine and Molecular Science, Gunma University Graduate School of Medicine, Maebashi, Japan
    Endocr J 58:287-96. 2011
    Tumors in multiple endocrine neoplasia type 1 (MEN1) are generally benign...
  81. pmc Total parathyroidectomy in a large cohort of cases with hyperparathyroidism associated with multiple endocrine neoplasia type 1: experience from a single academic center
    Fabio Luiz de Menezes Montenegro
    Department of Surgery, Head and Neck Surgery Section, Hospital das Clinicas, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil
    Clinics (Sao Paulo) 67:131-9. 2012
    ..Conversely, hyperparathyroidism associated with multiple endocrine neoplasia type 1 (hyperparathyroidism/multiple endocrine neoplasia type 1) is an asynchronic, asymmetrical ..
  82. doi Novel mutations in MEN1, CDKN1B and AIP genes in patients with multiple endocrine neoplasia type 1 syndrome in Spain
    Oihana Belar
    Endocrinology Research Group, Cruces Hospital, CIBERER, Barakaldo, Bizkaia, Spain
    Clin Endocrinol (Oxf) 76:719-24. 2012
    b>Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant disorder mostly owing to a genetic defect in MEN1 gene. Not all patients with MEN1 phenotype present a defect in this gene...
  83. doi EUS-FNA for pancreatic neuroendocrine tumors: a tertiary cancer center experience
    Muslim Atiq
    Department of Gastroenterology, MD Anderson Cancer Center, 1515 Holcombe Blvd, Unit 1466, Houston, TX 77030, USA
    Dig Dis Sci 57:791-800. 2012
    ..With this background, we decided to review our experience from a tertiary cancer center with regard to the presentation and clinical features of PNET and the diagnostic utility of EUS-FNA in this scenario...
  84. pmc Cushing's syndrome in multiple endocrine neoplasia type 1
    William F Simonds
    Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, 10 Center Drive, Bethesda, MD 20892, USA
    Clin Endocrinol (Oxf) 76:379-86. 2012
    In patients with multiple endocrine neoplasia type 1 (MEN1), Cushing's syndrome (CS) from endogenous hypercortisolism can result from pituitary, adrenal or other endocrine tumours...
  85. doi Concurrent primary hyperparathyroidism and humoral hypercalcemia of malignancy in a patient with multiple endocrine neoplasia type 1
    Anna Milanesi
    Division of Endocrinology, Samuel Oschin Comprehensive Cancer Institute, Cedars Sinai Medical Center, Los Angeles, CA 90048, USA
    Pancreas 40:634-7. 2011
    We report a patient with multiple endocrine neoplasia type 1 presenting with elevation of parathyroid hormone-related protein (PTHrP) from a metastatic pancreatic neuroendocrine tumor (PNET), and parathyroid hormone (PTH) from primary ..
  86. doi Multiple endocrine neoplasia type 1-associated thyrotropin-producing pituitary carcinoma: report of a probable de novo example
    Bernd W Scheithauer
    Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Hum Pathol 40:270-8. 2009
    ..and morphologically well-documented example of pituitary thyrotropin cell carcinoma in a man with multiple endocrine neoplasia type 1 syndrome...
  87. pmc Biochemical, bone and renal patterns in hyperparathyroidism associated with multiple endocrine neoplasia type 1
    Delmar M Lourenço
    Endocrine Genetics Unit, Division of Endocrinology, Hospital das Clinicas, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil
    Clinics (Sao Paulo) 67:99-108. 2012
    Primary hyperparathyroidism associated with multiple endocrine neoplasia type I (hyperparathyroidism/multiple endocrine neoplasia type 1) differs in many aspects from sporadic hyperparathyroidism, which is the most frequently occurring ..
  88. pmc Surgical treatment of pancreatic endocrine tumors in multiple endocrine neoplasia type 1
    Marcel Cerqueira Cesar Machado
    Department of Surgery, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil
    Clinics (Sao Paulo) 67:145-8. 2012
    Surgical approaches to pancreatic endocrine tumors associated with multiple endocrine neoplasia type 1 may differ greatly from those applied to sporadic pancreatic endocrine tumors...
  89. ncbi Patients with multiple endocrine neoplasia type 1 with gastrinomas have an increased risk of severe esophageal disease including stricture and the premalignant condition, Barrett's esophagus
    K Martin Hoffmann
    Digestive National Institutes of Health National Institute of Diabetes and Digestive and Kidney Diseases Digestive Diseases Branch, Bethesda, Maryland 20892 1804, USA
    J Clin Endocrinol Metab 91:204-12. 2006
    b>Multiple endocrine neoplasia type 1 (MEN1) patients frequently develop Zollinger-Ellison syndrome (MEN1/ZES)...
  90. ncbi Studies of the murine homolog of the multiple endocrine neoplasia type 1 (MEN1) gene, men1
    J H Bassett
    MRC Molecular Endocrinology Group, MRC Clinical Sciences Center, Imperial College School of Medicine, Hammersmith Hospital, London, United Kingdom
    J Bone Miner Res 14:3-10. 1999
    The murine homolog of the multiple endocrine neoplasia type 1 (MEN1) gene (men1), which in humans is associated with tumors of the parathyroids, pancreas, and pituitary, has been characterized by isolating 27 clones from a mouse ..
  91. doi Predicting the risk of multiple endocrine neoplasia type 1 for patients with commonly occurring endocrine tumors
    Joanne M de Laat
    Department of Internal Medicine, L00 408, University Medical Center Utrecht, PO Box 85500, 3508 GA Utrecht, The Netherlands
    Eur J Endocrinol 167:181-7. 2012
    Endocrine diseases that can be part of the rare inheritable syndrome multiple endocrine neoplasia type 1 (MEN1) commonly occur in the general population...
  92. ncbi Serum gastrin in Zollinger-Ellison syndrome: II. Prospective study of gastrin provocative testing in 293 patients from the National Institutes of Health and comparison with 537 cases from the literature. evaluation of diagnostic criteria, proposal of new
    Marc J Berna
    Digestive Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland 20892 1804, USA
    Medicine (Baltimore) 85:331-64. 2006
    ..For secretin testing, the criterion with the highest sensitivity and specificity is an increase of > or =120 pg/mL, which should replace other criteria commonly used today...
  93. ncbi Transcription regulation of the multiple endocrine neoplasia type 1 gene in human and mouse
    Barbara Zablewska
    Department of Molecular Medicine, Karolinska Institutet, S 17176 Stockholm, Sweden
    J Clin Endocrinol Metab 88:3845-51. 2003
    ..Our data confirm that the expression of the MEN1 gene is regulated by a feedback from its product menin...
  94. pmc Pathogenesis of gastrinomas associated with multiple endocrine neoplasia type 1
    D M Pritchard
    Division of Gastroenterology, School of Clinical Sciences, University of Liverpool, The Henry Wellcome Laboratory, Nuffield Building, Crown Street, Liverpool L69 3GE, UK
    Gut 56:606-7. 2007
  95. pmc Allelic deletion of the MEN1 gene in duodenal gastrin and somatostatin cell neoplasms and their precursor lesions
    M Anlauf
    Department of Pathology W, University of Kiel, Michaelisstr 11, 24105 Kiel, Germany
    Gut 56:637-44. 2007
    Patients with a multiple endocrine neoplasia type 1 (MEN1)-associated Zollinger-Ellison syndrome (ZES) show multifocal duodenal gastrinomas and precursor lesions.
  96. doi Assessment of p27 (cyclin-dependent kinase inhibitor 1B) and aryl hydrocarbon receptor-interacting protein (AIP) genes in multiple endocrine neoplasia (MEN1) syndrome patients without any detectable MEN1 gene mutations
    Susana Igreja
    Department of Endocrinology, Barts and the London School of Medicine, London, UK
    Clin Endocrinol (Oxf) 70:259-64. 2009
    ..The objective of this study was to evaluate the possible contribution of CDKN1B and AIP germline mutations in a cohort of MEN1 mutation-negative MEN1 syndrome patients...
  97. doi Role of multiple endocrine neoplasia type 1 mutational analysis in clinical practice
    Paul J Newey
    Academic Endocrine Unit, Nuffield Department of Clinical Medicine, University of Oxford, Churchill Hospital, Oxford, United Kingdom
    Endocr Pract 17:8-17. 2011
    ..To review and assess the role of MEN1 mutational analysis in clinical practice...
  98. doi Presentation and outcome of pancreaticoduodenal endocrine tumors in multiple endocrine neoplasia type 1 syndrome
    Maria Vittoria Davì
    Department of Medicine, University of Verona, Italy mariavittoria davi ospedaleuniverona it
    Neuroendocrinology 94:58-65. 2011
    To assess presentation and outcome of pancreaticoduodenal endocrine tumors (PDETs) in a single center series of multiple endocrine neoplasia type 1 (MEN1) patients.
  99. ncbi Primary structure, gene expression and chromosomal mapping of rodent homologs of the MEN1 tumor suppressor gene
    W Karges
    Department of Internal Medicine, University of Ulm, Germany
    Biochim Biophys Acta 1446:286-94. 1999
    Mutations of the MEN1 tumor suppressor gene cause the multiple endocrine neoplasia type 1 (MEN1) syndrome in humans, and they are involved in a variety of sporadic human endocrine tumors...
  100. ncbi Primary hyperparathyroidism in multiple endocrine neoplasia type 1: individualized management with low recurrence rates
    Chen Hsen Lee
    Department of Surgery, Taipei Veterans General Hospital, 201, Sec 2, Shih Pai Rd, Taipei, Taiwan
    Ann Surg Oncol 13:103-9. 2006
    To evaluate the outcomes in different surgical modalities for primary hyperparathyroidism in multiple endocrine neoplasia type 1 (MEN1) patients, intraoperative findings from a single surgeon were studied to investigate a potentially ..
  101. pmc Surgical approach in patients with hyperparathyroidism in multiple endocrine neoplasia type 1: total versus partial parathyroidectomy
    Francesco Tonelli
    Surgical Unit, Department of Clinical Physiopathology, University of Florence, Florence, Italy
    Clinics (Sao Paulo) 67:155-60. 2012
    Usually, primary hyperparathyroidism is the first endocrinopathy to be diagnosed in patients with multiple endocrine neoplasia type 1, and is also the most common one...

Research Grants27

  1. Link of beta cell proliferation and type 2 diabetes to epigenetic regulation
    Xianxin Hua; Fiscal Year: 2013
    ..Mutations in the multiple endocrine neoplasia type 1 gene (Men1), which encodes the nuclear protein menin, usually result in benign hyperplasia in ..
  2. Link of beta cell proliferation and type 2 diabetes to epigenetic regulation.
    Xianxin Hua; Fiscal Year: 2010
    ..Mutations in the multiple endocrine neoplasia type 1 gene (Men1), which encodes the nuclear protein menin, usually result in benign hyperplasia in ..
  3. Beta Cell Regeneration by an Epigenetic Pathway
    Xianxin Hua; Fiscal Year: 2013
    ..In this regard, mutations in the multiple endocrine neoplasia type 1 gene (MEN1), which encodes the nuclear protein menin, is the only genetically proven means to ..
  4. Development of novel anti-leukemia agents targeting the menin-MLL interaction
    Jolanta Grembecka; Fiscal Year: 2013
    ..If successful, our studies will result in compounds which may provide a novel therapeutic approach for the treatment of leukemias with MLL translocations. ..
  5. Men1 Control of Endocrine Cell Growth and Differentiation
    Seung K Kim; Fiscal Year: 2010
    ....
  6. Interactions of the angiopoietin and PD-ECGF pathways in tumor angiogenesis
    Edward L Schwartz; Fiscal Year: 2013
    ....
  7. Deciphering the Tissue Specificity of MEN1 Related Tumorigenesis
    STEVEN KENNETH LIBUTTI; Fiscal Year: 2013
    ..b>Multiple Endocrine Neoplasia type 1 (MEN1), a familial cancer syndrome characterized by tissue-restricted tumorigenesis, provides a ..
  8. The RBP2/JARID1A/KDM5A Histone Demethylase as a Potential Drug Target in Cancer
    William G Kaelin; Fiscal Year: 2013
    ..This proposal might establish that inhibiting RBP2 histone demethylase activity is a potential anticancer strategy and provide tools for developing drugs that target RBP2 or perhaps other enzymes of this class ..
  9. ISOLATION OF THE GENE FOR MULTIPLE ENDOCRINE NEOPLASIA 1
    Allen Bale; Fiscal Year: 1993
    ..b>Multiple endocrine neoplasia type 1 (MEN1), characterized by parathyroid hyperplasia and neoplasms of the pituitary and pancreatic ..
  10. Functional analysis of MEN1 in Drosophila model
    Allen Bale; Fiscal Year: 2004
    ..Determining which genetic pathways are important in menin-related neoplasia wilt lay the groundwork for rational medical therapy. ..
  11. PHYSICAL MAP & ORDERED COSMID SET: HUMAN CHROMOSOME 11
    GLEN EVANS; Fiscal Year: 1990
    ..the localization of important disease genes, such as those responsible for ataxia telangiectasia, multiple endocrine neoplasia Type 1, tuberous sclerosis, peripheral neuroepithelioma, and others...
  12. Molecular Mechanims of the Menin Tumor Suppressor Gene
    Mark Sawicki; Fiscal Year: 2002
    ..2. Characterize the function of menin as a DDKI (Dbf4 dependent kinase inhibitor). 3. Identify physiological factors and their underlying molecular mechanisms important for modulating menin DDK1 function. ..
  13. Menin regulation of pancreatic islet growth and fates
    SATYAJIT KARNIK; Fiscal Year: 2007
    The gene responsible for causing the human disease multiple endocrine neoplasia type 1 (MEN1), menin, is being investigated...
  14. Inhibitors of MLL-Menin Interaction
    Michael Cleary; Fiscal Year: 2007
    ..Thus, there is an urgent need for novel therapeutic modalities based on rational drug design targeting the molecular abnormalities that underlie leukemia pathogenesis. [unreadable] [unreadable] [unreadable]..
  15. Histone methylation and transcriptional by the menin tumor suppresor
    Joshua Francis; Fiscal Year: 2009
    ..The long-term goal of these studies are to identify therapeutic targets within the cell that could be specifically inhibited to control cellular proliferation. ..
  16. Influence of Tumor Immunogenicity to Immunotherapy
    Jeffrey Norton; Fiscal Year: 2006
    ..unreadable] [unreadable]..
  17. CLONING & ANALYSIS OF NOVEL TGF-BETA SIGNALING MOLECULES
    Xianxin Hua; Fiscal Year: 2002
    ..The novel cDNAs isolated by the antisense approach will be tested for its potential role as tumor suppressor genes. ..
  18. TGFBeta Signaling Mechanisms in the Pancreas
    Diane Simeone; Fiscal Year: 2003
    ..This grant proposal is submitted for the Small Grants Program for K08 Recipients-NIH K08 DK02637-01. ..
  19. TGFB SIGNALING PATHWAY IN THE PANCREAS
    Diane Simeone; Fiscal Year: 2003
    ..abstract_text> ..
  20. Epithelial-stromal cell interactions in breast cancer
    Kornelia Polyak; Fiscal Year: 2010
    ....
  21. Function of tumor suppressor gene menin
    Xianxin Hua; Fiscal Year: 2008
    ..abstract_text> ..
  22. Molecular Mechanims of the Menin Tumor Suppressor Gene
    Mark Sawicki; Fiscal Year: 2004
    ..2. Characterize the function of menin as a DDKI (Dbf4 dependent kinase inhibitor). 3. Identify physiological factors and their underlying molecular mechanisms important for modulating menin DDKI function...
  23. Cathartic-Free Dual-Energy CT Colonography
    Jeff Fidler; Fiscal Year: 2006
    ..The investigators have world-class expertise and resources in CT colonography, dual energy algorithms, and CT system calibrations. [unreadable] [unreadable]..
  24. HIN-1,A Novel Putative Breast Tumor Suppressor Gene
    Kornelia Polyak; Fiscal Year: 2006
    ..abstract_text> ..
  25. Studies of Pancreatic TGFBeta-Mediated Signaling
    Diane Simeone; Fiscal Year: 2007
    ..The long term goal of this project is to gain a detailed understanding of TGF signaling mechanisms in the pancreas that may be of benefit in the treatment and/or prevention of human pancreatic disease. ..
  26. Regulation of apoptosis by menin
    Xianxin Hua; Fiscal Year: 2009
    ..abstract_text> ..