Download Gene Section MN1 (meningioma 1) Atlas of Genetics and Cytogenetics

Atlas of Genetics and Cytogenetics
in Oncology and Haematology
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Gene Section
Short Communication
MN1 (meningioma 1)
Jean-Loup Huret
Genetics, Department of Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021
Poitiers, France
Published in Atlas Database: October 1997
Online version is available at: http://AtlasGeneticsOncology.org/Genes/MN1.html
DOI: 10.4267/2042/32044
This work is licensed under a Creative Commons Attribution-Non-commercial-No Derivative Works 2.0 France Licence.
© 1997 Atlas of Genetics and Cytogenetics in Oncology and Haematology
Prognosis
Yet uncertain; median survival 2 yrs.
Cytogenetics
Additional anomalies: +8.
Hybrid/Mutated Gene
5’ MN1 - 3’ ETV6.
Abnormal Protein
N-term MN1 and most of it, comprising the
glutamine/proline rich domain, fused to the DNA
binding of ETV6 in C-term; nuclear protein.
Oncogenesis
May act as an altered transcription factor.
Identity
Location: 22q11-12
DNA/RNA
Description
2 exons at least, the first being very CG rich and with
CAG repeats; spans about 70 kb.
Transcription
Alternate splicing: 4.5 and 8 kb mRNA; coding
sequence: 4.0 kb.
Protein
Meningioma (some of them)
Description
References
1319 and 1342 amino acids; glutamine and prolin rich
(may function as transactivation domains).
Buijs A, Sherr S, van Baal S, van Bezouw S, van der Plas D,
Geurts van Kessel A, Riegman P, Lekanne Deprez R,
Zwarthoff E, Hagemeijer A, et al. Translocation
(12;22)(p13;q11) in myeloproliferative disorders results in
fusion of the ETS-like TEL gene on 12p13 to the MN1 gene on
22q11. Oncogene 1995 Apr 20;10(8):1511-9. Erratum in
Oncogene 1995 Aug 17;11(4):809.
Expression
Ubiquitously expressed; high expression in muscle, low
otherwise.
Function
Lekanne Deprez RH, Riegman PH, Groen NA, Warringa UL,
van Biezen NA, Molijn AC, Bootsma D, de Jong PJ, Menon
AG, Kley NA, et al. Cloning and characterization of MN1, a
gene from chromosome 22q11, which is disrupted by a
balanced translocation in a meningioma. Oncogene 1995 Apr
20;10(8):1521-8.
Transcriptional regulator.
Implicated in
t(12;22)(p13;q11)/myeloid malignancies
→ MN1/ETV6
This article should be referenced as such:
Huret JL. MN1 (meningioma 1). Atlas Genet Cytogenet Oncol
Haematol.1997;1(2):48.
Disease
M4 ANLL and other myeloid malignancies (MDS...).
Atlas Genet Cytogenet Oncol Haematol. 1997;1(2)
48