Atlas of Genetics and Cytogenetics in Oncology and Haematology
MTCP1 (Mature T Cell Proliferation 1)
| Identity |
| Other names | C6.1B |
| HGNC (Hugo) | MTCP1 |
| Location | Xq28 |
| Location_base_pair | Starts at 154292309 and ends at 154299547 bp from pter ( according to hg19-Feb_2009) [Mapping] |
| Local_order | centromere- Factor VIII - c6.1A - MTCP1 - telomere |
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| MTCP1 (Xq28) - Courtesy Mariano Rocchi, Resources for Molecular Cytogenetics. Laboratories willing to validate the probes are welcome : contact rocchi@biologia.uniba.it | |
| DNA/RNA |
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| DNA Diagram | |
| Description | 7 exons; about 10 kb |
| Transcription | complex; alternative splicing: two donor sites in exon 1; transcripts A, the most abondant, ubiquitous: splicing from exon 1 to exon 6; transcripts B, rare: splicing from exon 1 to exon 2; initiation of the transcription: an alternative site of initiation of the transcription in intron 1 has been found in one tumor with a translocation breakpoint in intron 1 |
| Protein |
| Description | p8 MTCP1: coded by transcripts A, 68 amino acids; one domain formed by 3 alpha helices held together by two disulphide bridges in an antiparallel coiled-coil motif p13 MTCP1: coded by transcripts B, 107 amino acides; one domain with a b-barrel topology |
| Expression | ubiquitously expressed protein expression undetectable in physiological conditions |
| Localisation | mitochondrial cytosol |
| Function | unknown unknown |
| Homology | none TCL1 (39% identity, similar tridimentional structure) |
| Implicated in |
| Entity | t(X;14)(q28;q12) / prolymphocytic leukaemia --> TCRA/D - MTCP1 |
| Disease | T-cell prolymphocytic leukaemia |
| Cytogenetics | associated with i(8q) |
| Hybrid/Mutated Gene | unconstant TCRA-c6.1A transcripts have been described |
| Abnormal Protein | none |
| Oncogenesis | over expression of p13 MTCP1 is considered as critical in the oncogenetic mechanism |
| Entity | t(X;7)(q28;q35) / prolymphocytic leukaemia --> TCRB - MTCP1 |
| Disease | T-cell prolymphocytic leukemia |
| Oncogenesis | over expression of p13 MTCP1 |
| External links |
| Bibliography |
| The chromosomal translocation t(X;14)(q28;q11) in T-cell pro-lymphocytic leukaemia breaks within one gene and activates another. |
| Fisch P, Forster A, Sherrington PD, Dyer MJ, Rabbitts TH |
| Oncogene. 1993 ; 8 (12) : 3271-3276. |
| PMID 8247530 |
| The MTCP-1/c6.1B gene encodes for a cytoplasmic 8 kD protein overexpressed in T cell leukemia bearing a t(X;14) translocation. |
| Soulier J, Madani A, Cacheux V, Rosenzwajg M, Sigaux F, Stern MH |
| Oncogene. 1994 ; 9 (12) : 3565-3570. |
| PMID 7970717 |
| A gene on chromosome Xq28 associated with T-cell prolymphocytic leukemia in two patients with ataxia telangiectasia. |
| Thick J, Mak YF, Metcalfe J, Beatty D, Taylor AM |
| Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1994 ; 8 (4) : 564-573. |
| PMID 8152252 |
| Expression of p13MTCP1 is restricted to mature T-cell proliferations with t(X;14) translocations. |
| Madani A, Choukroun V, Soulier J, Cacheux V, Claisse JF, Valensi F, Daliphard S, Cazin B, Levy V, Leblond V, Daniel MT, Sigaux F, Stern MH |
| Blood. 1996 ; 87 (5) : 1923-1927. |
| PMID 8634440 |
| Expression of either the TCL1 oncogene, or transcripts from its homologue MTCP1/c6.1B, in leukaemic and non-leukaemic T cells from ataxia telangiectasia patients. |
| Thick J, Metcalfe JA, Mak YF, Beatty D, Minegishi M, Dyer MJ, Lucas G, Taylor AM |
| Oncogene. 1996 ; 12 (2) : 379-386. |
| PMID 8570215 |
| Solution structure of human p8MTCP1, a cysteine-rich protein encoded by the MTCP1 oncogene, reveals a new alpha-helical assembly motif. |
| Barthe P, Yang YS, Chiche L, Hoh F, Strub MP, Guignard L, Soulier J, Stern MH, van Tilbeurgh H, Lhoste JM, Roumestand C |
| Journal of molecular biology. 1997 ; 274 (5) : 801-815. |
| PMID 9405159 |
| Alternative origin of p13MTCP1-encoding transcripts in mature T-cell proliferations with t(X;14) translocations. |
| Gritti C, Choukroun V, Soulier J, Madani A, Dastot H, Leblond V, Radford-Weiss I, Valensi F, Varet B, Sigaux F, Stern MH |
| Oncogene. 1997 ; 15 (11) : 1329-1335. |
| PMID 9315101 |
| Solution structure of the recombinant human oncoprotein p13MTCP1. |
| Yang YS, Guignard L, Padilla A, Hoh F, Strub MP, Stern MH, Lhoste JM, Roumestand C |
| Journal of biomolecular NMR. 1998 ; 11 (3) : 337-354. |
| PMID 9691281 |
| REVIEW articles | automatic search in PubMed |
| Last year publications | automatic search in PubMed |
| Contributor(s) |
| Written | 03-1998 | Marc-Henri Stern |
| Unité INSERM U462, Centre Hayem, Hopital Saint Louis, 75475 Paris Cedex 10, France |
| Citation |
| This paper should be referenced as such : |
| Stern MH . MTCP1 (Mature T Cell Proliferation 1). Atlas Genet Cytogenet Oncol Haematol. March 1998 . URL : http://AtlasGeneticsOncology.org/Genes/MTCP1ID89.html |
| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Thu Jul 15 14:46:47 CEST 2010 |
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