keyword
MENU ▼
Read by QxMD icon Read
search

MEN1

keyword
https://www.readbyqxmd.com/read/28943239/how-to-effectively-treat-acute-leukemia-patients-bearing-mll-rearrangements
#1
REVIEW
Dieter Steinhilber, Rolf Marschalek
Chromosomal translocations - leading to the expression of fusion genes - are well-studied genetic abberrations associated with the development of leukemias. Most of them represent altered transcription factors that affect transcription or epigenetics, while others - like BCR-ABL - are enhancing signaling. BCR-ABL has become the prototype for rational drug design, and drugs like Imatinib and subsequently improved drugs have a great impact on cancer treatments. By contrast, MLL-translocations in acute leukemia patients are hard to treat, display a high relapse rate and the overall survival rate is still very poor...
September 21, 2017: Biochemical Pharmacology
https://www.readbyqxmd.com/read/28940393/clinical-features-and-prognosis-of-thymic-neuroendocrine-tumors-associated-with-multiple-endocrine-neoplasia-type-1-a-single-center-study-systematic-review-and-meta-analysis
#2
Lei Ye, Weixi Wang, Naykky Singh Ospina, Lei Jiang, Ioannis Christakis, Jieli Lu, Yulin Zhou, Wei Zhu, Yanan Cao, Shu Wang, Nancy D Perrier, William F Young, Guang Ning, Weiqing Wang
OBJECTIVE: Thymic neuroendocrine tumor (TH-NET) accounts for almost 20% of multiple endocrine neoplasia type 1 (MEN1)-associated mortality. Identifying risk factors for the development of these rare tumors and prognostic factors for clinical outcomes will be helpful in clinical practice. DESIGN AND PATIENTS: We performed a retrospective analysis of patients treated for TH-NET associated with MEN1 in a single institution and meta-analysis of literature reports. We used a fixed effect model to pool results across studies to evaluate the prevalence, clinical features, and prognosis...
September 20, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28938468/long-term-natural-course-of-small-non-functional-pancreatic-neuroendocrine-tumors-in-men1-results-from-the-dmsg
#3
Carolina R C Pieterman, Joanne M de Laat, Jos W R Twisk, Rachel S van Leeuwaarde, Wouter W de Herder, Koen M A Dreijerink, Ad R M M Hermus, Olaf M Dekkers, Anouk N A van der Horst-Schrivers, Madeleine L Drent, Peter H Bisschop, Bastiaan Havekes, Inne H M Borel Rinkes, Menno R Vriens, Gerlof D Valk
Background: Pancreatic neuroendocrine tumors (pNETs) are highly prevalent in patients with Multiple Endocrine Neoplasia type 1 (MEN1) and metastatic disease is an important cause of MEN1-related mortality. Especially small non-functional (NF) pNETs pose a challenge to the treating physician and more information is needed regarding their natural course. We assessed long-term natural history of small NF-pNETs and its modifiers in the Dutch MEN1 population. Patients and Methods: Retrospective longitudinal observational cohort study of patients with small (<2cm) NF-pNETs from the Dutch national MEN1 database which includes >90% of the Dutch MEN1 population...
August 4, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28924486/expression-of-transcription-factors-in-men1-associated-pancreatic-neuroendocrine-tumors
#4
Yasutaka Takeda, Yukihiro Fujita, Kentaro Sakai, Tomoe Abe, Tomonobu Nakamura, Tsuyoshi Yanagimachi, Hidemitsu Sakagami, Jun Honjo, Atsuko Abiko, Yuichi Makino, Masakazu Haneda
MEN1-associated pancreatic neuroendocrine tumors (pNETs) may potentially express distinct hormones, but the mechanism has not been elucidated. Transcription factors such as MafA and Pdx1 have been identified to lead to beta cell differentiation, while Arx and Brn4 to alpha cell differentiation in developing pancreas. We hypothesized those transcription factors are important to produce specific hormones in pNETs, similarly to developing pancreas, and examined the expression of transcription factors in a case of MEN1 who showed immunohistological coexistence of several hormone-producing pNETs including insulinoma...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28902384/-multiple-endocrine-neoplasia
#5
Ludwig Schaaf, Friedhelm Raue
Multiple endocrine neoplasia type 1 and 2 are hereditary cancer syndromes. They are characterized by the occurrence of many benign and malignant tumor types, in MEN1 parathyroid tumors, pituitary tumors, and pancreas tumors, in MEN2 medullary thyroid carcinoma, pheochromocytoma, and parathyroid tumors. The autosomal dominant inherited tumor syndromes are caused by mutations in the MEN1 gene, a tumor suppressor gene, and mutations in the RET gene, an activated oncogene, in MEN2. The clinical expression of the different tumors can vary within and between families, with a good genotype-phenotype correlation in MEN2...
September 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28899949/the-future-genetics-advances-in-men1-therapeutic-approaches-and-management-strategies
#6
REVIEW
Sunita K Agarwal
The identification of the multiple endocrine neoplasia type 1 (MEN1) gene in 1997 has shown that germline heterozygous mutations in the MEN1 gene located on chromosome 11q13 predisposes to the development of tumors in the MEN1 syndrome. Tumor development occurs upon loss of the remaining normal copy of the MEN1 gene in MEN1-target tissues. Therefore, MEN1 is a classic tumor suppressor gene in the context of MEN1. This tumor suppressor role of the protein encoded by the MEN1 gene, menin, holds true in mouse models with germline heterozygous Men1 loss, wherein MEN1-associated tumors develop in adult mice after spontaneous loss of the remaining non-targeted copy of the Men1 gene...
October 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28899948/happy-20th-anniversary-men1-from-positional-cloning-to-gene-function-restoration
#7
EDITORIAL
Frank Weber, Lois M Mulligan
No abstract text is available yet for this article.
October 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28881068/molecular-genetics-of-syndromic-and-non-syndromic-forms-of-parathyroid-carcinoma
#8
REVIEW
Luís Cardoso, Mark Stevenson, Rajesh V Thakker
Parathyroid carcinoma (PC) may occur as part of a complex hereditary syndrome or an isolated (i.e., non-syndromic) non-hereditary (i.e., sporadic) endocrinopathy. Studies of hereditary and syndromic forms of PC, which include the hyperparathyroidism-jaw tumor syndrome (HPT-JT), multiple endocrine neoplasia types 1 and 2 (MEN1 and MEN2), and familial isolated primary hyperparathyroidism (FIHP), have revealed some genetic mechanisms underlying PC. Thus, cell division cycle 73 (CDC73) germline mutations cause HPT-JT, and CDC73 mutations occur in 70% of sporadic PC, but in only ∼2% of parathyroid adenomas...
September 7, 2017: Human Mutation
https://www.readbyqxmd.com/read/28874394/looking-beyond-the-thyroid-advances-in-understanding-of-pheochromocytoma-and-hyperparathyroidism-phenotypes-in-men2-and-of-non-men2-familial-forms
#9
Carole Guerin, Pauline Romanet, David Taieb, Thierry Brue, André Lacroix, Frederic Sebag, Anne Barlier, Frederic Castinetti
Over the last years, the knowledge of MEN2 and non MEN2 familial forms of pheochromocytoma (PHEO) has increased. In MEN2, PHEO is the second most frequent disease: the penetrance and age at diagnosis depend on the mutation of RET. Given the prevalence of bilateral PHEO (50% by age 50), adrenal sparing surgery, aimed at sparing a part of the adrenal cortex to avoid adrenal insufficiency, should be systematically considered in patients with bilateral PHEO. Non MEN2 familial forms of PHEO now include more than 20 genes: however, only small phenotypic series have been reported, suggesting that phenotypic features of isolated hereditary PHEO must be better explored, and follow-up series are needed to better understand the outcome of patients carrying mutations of these genes...
September 5, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28870973/primary-hyperparathyroidism-in-young-patients-in-russia-high-frequency-of-hyperparathyroidism-jaw-tumour-syndrome
#10
Elizaveta Mamedova, Natalya Mokrysheva, Evgeny V Vasilyev, Vasiliy Petrov, Ekaterina Pigarova, Sergey Kuznetsov, Nikolay Kuznetsov, Liudmila Rozhinskaya, Galina A Melnichenko, Ivan Dedov, Anatoly Tiulpakov
BACKGROUND: Primary hyperparathyroidism (PHPT) is a relatively rare disorder among children, adolescents and young adults. Its development at an early age is suspicious for hereditary causes, though the need for routine genetic testing remains controversial. OBJECTIVE: To identify and describe hereditary forms of PHPT in patients with manifestation of the disease under 40 years of age. DESIGN: We enrolled 65 patients with PHPT diagnosed before 40 years of age...
September 4, 2017: Endocrine Connections
https://www.readbyqxmd.com/read/28859856/gastrin-induces-nuclear-export-and-proteasome-degradation-of-menin-in-enteric-glial-cells
#11
Sinju Sundaresan, Cameron A Meininger, Anthony J Kang, Amanda L Photenhauer, Michael M Hayes, Nirakar Sahoo, Jolanta Grembecka, Tomasz Cierpicki, Lin Ding, Thomas J Giordano, Tobias Else, David J Madrigal, Malcolm J Low, Fiona Campbell, Ann-Marie Baker, Haoxing Xu, Nicholas A Wright, Juanita L Merchant
BACKGROUND & AIMS: The multiple endocrine neoplasia, type 1 (MEN1) locus encodes the nuclear protein and tumor suppressor menin. MEN1 mutations frequently cause neuroendocrine tumors (NETs) such as gastrinomas, characterized by their predominant duodenal location and local metastasis at time of diagnosis. Diffuse gastrin cell hyperplasia precedes the appearance of MEN1 gastrinomas, which develop within submucosal Brunner's glands. We investigated how menin regulates expression of the gastrin gene and induces generation of submucosal gastrin-expressing cell hyperplasia...
August 28, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28833384/primary-hyperparathyroidism-in-chinese-children-and-adolescents-a-single-center-experience-at-peking-union-medical-college-hospital
#12
Wenbo Wang, Jing Kong, Min Nie, Yan Jiang, Mei Li, Weibo Xia, Xunwu Meng, Xiaoping Xing, Ou Wang
OBJECTIVE: Primary hyperparathyroidism (PHPT) in children is thought to be extremely rare. Our study aimed to summarize the clinical characteristics and the molecular genetics in patients with pediatric PHPT in China. DESIGN: Retrospective observational study. METHODS: A total of 59 pediatric PHPT patients (onset age <18 years) admitted to Peking Union Medical College Hospital from 1975 to 2015 were retrospectively identified. A group of 118 adult PHPT patients who presented during the same period were selected for comparing clinical characteristics between the two groups...
August 22, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28811300/epigenetic-regulation-by-the-menin-pathway
#13
REVIEW
Zijie Feng, Jian Ma, Xianxin Hua
There is a trend of increasing prevalence of neuroendocrine tumors (NETs), and the inherited multiple endocrine neoplasia type 1 (MEN1) syndrome serves as a genetic model to investigate how NETs develop and the underlying mechanisms. Menin, encoded by the MEN1 gene, at least partly acts as a scaffold protein by interacting with multiple partners to regulate cellular homeostasis of various endocrine organs. Menin has multiple functions including regulation of several important signaling pathways by controlling gene transcription...
October 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28811299/twenty-years-of-menin-emerging-opportunities-for-restoration-of-transcriptional-regulation-in-men1
#14
REVIEW
Koen M A Dreijerink, H T Marc Timmers, Myles Brown
Since the discovery of the multiple endocrine neoplasia type 1 (MEN1) gene in 1997, elucidation of the molecular function of its protein product, menin, has been a challenge. Biochemical, proteomics, genetics and genomics approaches have identified various potential roles, which converge on gene expression regulation. The most consistent findings show that menin connects transcription factors and chromatin-modifying enzymes, in particular, the histone H3K4 methyltransferase complexes MLL1 and MLL2. Chromatin immunoprecipitation combined with next-generation sequencing has enabled studying genome-wide dynamics of chromatin binding by menin...
October 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28811298/the-future-surgical-advances-in-men1-therapeutic-approaches-and-management-strategies
#15
Samira Mercedes Sadowski, Guillaume Cadiot, Eric Dansin, Pierre Goudet, Frédéric Triponez
Multiple endocrine neoplasia type 1 (MEN1) is a hereditary autosomal dominant disorder associated with numerous neuroendocrine tumors (NETs). Recent advances in the management of MEN1 have led to a decrease in mortality due to excess hormones; however, they have also led to an increase in mortality from malignancy, particularly NETs. The main challenges are to localize these tumors, to select those that need therapy because of the risk of aggressive behavior, and to select the appropriate therapy associated with minimal morbidity...
August 15, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28811081/prognostic-value-of-who-grade-in-pancreatic-neuro-endocrine-tumors-in-multiple-endocrine-neoplasia-type-1-results-from-the-dutchmen1-study-group
#16
Elfi B Conemans, Lodewijk A A Brosens, Gabriela M Raicu-Ionita, Carolina R C Pieterman, Wouter W de Herder, Olaf M Dekkers, Ad R Hermus, Anouk N van der Horst-Schrivers, Peter H Bisschop, Bas Havekes, Madeleine L Drent, H Th Marc Timmers, G Johan Offerhaus, Gerlof D Valk, Menno R Vriens
BACKGROUND: The prognostic value of WHO grade in pancreatic neuroendocrine tumors (PanNETs) in patients with Multiple Endocrine Neoplasia Type 1 (MEN1) is unknown. METHODS: We performed a cohort study using the Dutch National MEN1 database, which includes >90% of the Dutch MEN1 population with data collected between 1990 and 2014. Formalin-fixed paraffin embedded tissue blocks from the largest resected PanNET per patient were collected. MIB1 staining was performed and KI67 labeling index (LI) was determined by manual eye-counting under a microscope and by digital image analysis...
July 31, 2017: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
https://www.readbyqxmd.com/read/28803964/tgf%C3%AE-smad-signalling-modulates-mll-and-mll-af4-mediated-5-lipoxygenase-promoter-activation
#17
Meike J Saul, Florian Groher, Anett B Hegewald, Michaela Müller-McNicoll, Rolf Marschalek, Beatrix Suess, Dieter Steinhilber
5-Lipoxygenase (5-LO) catalyzes the initial two steps of the conversion of arachidonic acid to leukotrienes which represent a group of pro-inflammatory lipid mediators involved in immune defense reactions as well as inflammation, allergy and cancer. Transforming growth factor-β (TGFβ) and calcitriol strongly upregulate 5-LO expression during myeloid cell differentiation and MLL-AF4 has been shown to strongly activate the 5-LO promoter. Here, we investigated the role of TGFβ/SMAD signalling in 5-LO promoter activation...
August 10, 2017: Prostaglandins & Other Lipid Mediators
https://www.readbyqxmd.com/read/28790162/the-future-diagnostic-and-imaging-advances-in-men1-therapeutic-approaches-and-management-strategies
#18
REVIEW
Jerena Manoharan, Max B Albers, Detlef K Bartsch
Prospective randomized data are lacking, but current clinical expert guidelines recommend annual screening examinations, including laboratory assessments and various imaging modalities (e.g. CT, MRI, scintigraphy and EUS) for patients with multiple endocrine neoplasia type 1 (MEN1). Routine screening is proposed to detect and localize neuroendocrine manifestations as early as possible. The goal is timely intervention to improve quality of life and to increase life expectancy by preventing the development of life-threatening hormonal syndromes and/or metastatic disease...
October 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28782520/direct-interaction-of-menin-leads-to-ubiquitin-proteasomal-degradation-of-%C3%AE-catenin
#19
Byungho Kim, Tae-Yang Song, Kwan Young Jung, Seul Gi Kim, Eun-Jung Cho
Menin, encoded by the multiple endocrine neoplasia type 1 (MEN1) gene, is a tumor suppressor and transcription regulator. Menin interacts with various proteins as a scaffold protein and is proposed to play important roles in multiple physiological and pathological processes by controlling gene expression, proliferation, and apoptosis. The mechanisms underlying menin's suppression of tumorigenesis are largely elusive. In this study, we showed that menin was essential for the regulation of canonical Wnt/β-catenin signaling in cultured cells...
August 4, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28768698/the-future-advances-in-therapeutic-approach-and-management-strategies-for-men1
#20
Rachel S van Leeuwaarde, Joanne M de Laat, Carolina R C Pieterman, Koen M A Dreijerink, Menno Vriens, Gerlof D Valk
Multiple endocrine neoplasia type 1 is a rare autosomal inherited disorder associated with a high risk for patients to simultaneously develop tumours of the parathyroid glands, duodeno-pancreatic neuroendocrine tumors and tumors of the anterior pituitary gland. Early identification of MEN1 in patients enables presymptomatic screening of manifestations which makes timely interventions possible with the intention to prevent morbidity and mortality. Causes of death nowadays have shifted towards local or metastatic progression of malignant neuro endocrine tumors...
August 2, 2017: Endocrine-related Cancer
keyword
keyword
9482
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"