Written | 2010-01 | Xiao Wang, Birgitta Sander |
Department of Pathology, F46, Karolinska Institutet, SE 141 86 Stockholm, Sweden (XW); Department of Pathology, F46, Karolinska University Hospital, Karolinska Institutet, SE 141 86 Stockholm, Sweden (BS) |
Identity |
HGNC (Hugo) | SOX11 |
HGNC Alias name | SRY-related HMG-box gene 11 |
HGNC Previous name | SRY (sex determining region Y)-box 11 | SRY-box 11 |
LocusID (NCBI) | 6664 |
Atlas_Id | 42357 |
Location | 2p25.2 [Link to chromosome band 2p25] |
Location_base_pair | Starts at 5692384 and ends at 5701385 bp from pter ( according to GRCh38/hg38-Dec_2013) [Mapping SOX11.png] |
Fusion genes (updated 2017) | Data from Atlas, Mitelman, Cosmic Fusion, Fusion Cancer, TCGA fusion databases with official HUGO symbols (see references in chromosomal bands) |
SOX11 (2p25.2) / ACTL8 (1p36.13) | SOX11 (2p25.2) / PGK1 (Xq21.1) | SOX11 (2p25.2) / ZFP36L2 (2p21) | |
DNA/RNA |
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The human SOX11 gene. The only exon is indicated by the red box. The number above the box shows the size of the exon. | |
Description | The intronless gene encompasses 8718 base pairs and is located at 2p25.2. |
Transcription | The mRNA SOX11 transcript contains one exon with 8718 base pairs. |
Protein |
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The human SOX11 protein and functional domains. Numbers indicate the amino acid positions at the beginning and the end of each domain. The grey bar shows the High-Mobility-Group (HMG) box DNA-binding domain; and the dark blue bar shows the TransActivation Domain (TAD). | |
Description | The human SOX11 protein has 441 amino acids and 46.7 KDa molecular weight. It contains two functional domains: a Sry-related high-mobility group (HMG) box (Sox) DNA-binding domain, located in the N-terminal third (47-122), and a transactivation domain (TAD), located at the C-terminus (408-441) (Dy et al., 2008; Penzo-Méndez, 2009). |
Expression | SOX11 is widely expressed during organogenesis in the embryo and is highly expressed in the central and peripheral nervous system of the human fetus (Jay et al., 1995; Sock et al., 2004). SOX11 expression is more restricted by birth, and low transcript levels of SOX11 were detected in adult colon, small intestine, heart and brain (Weigle et al., 2005). High level of SOX11 mRNA is only present in normal prostate tissue (Brennan et al., 2009). |
Localisation | Nucleus. |
Function | SOX11 contains domains that may function as transcriptional activators or repressors and is a member of the group C SOX transcription factor family. It appears to have critical roles in embryonic neurogenesis and development of many organ systems including heart, palate and eyelids (Sock et al., 2004). SOX11 can, together with SOX4, regulate the differentiation of neuronal progenitors (Bergsland et al., 2006). SOX11 is involved in the transcriptional regulation of specific gene expression programs in adult neurogenesis at the stage of the immature neuron (Haslinger et al., 2009). SOX11 controls morphological maturation such as neurite growth (Jankowski et al., 2006) and modulates the regeneration following peripheral nerve injury (Jankowski et al., 2009). The class-III beta-tubulin gene TUBB3 is the first and still the unique gene to be identified as a direct target of SOX11 (Dy et al., 2008). SOX11 has been suggested to bind and regulate LINE retrotransposons (Tchénio, 2000). |
Homology | The two domains are highly conserved regions between species (from human to fly). In the SOX C group, consisting of SOX4, SOX11 and SOX12, the HMG box and C33 domains are more highly conserved among Sox4 orthologues and SOX11 orthologues than among SOX12. |
Implicated in |
Note | |
Entity | Malignant glioma |
Note | SOX11 was identified by screening an expression database for genes highly expressed in glioblastoma multiforme (Weigle et al., 2005). Overexpression of SOX11 can prevent tumorigenesis of glioma-initiating cells by inducing their neuronal differentiation (Hide et al., 2009). |
Disease | Malignant glioma comprises of the majority of primary brain tumors with 16800 new cases reported each year in USA. |
Prognosis | SOX11 can serve as a target antigen for glioma-directed cytotoxic T lymphocytes (CTLs), and this novel CTL epitope may serve as a suitable candidate for a T cell-based immunotherapy of glioma patients (Schmitz et al., 2007). |
Entity | Medulloblastoma |
Note | SOX11 has been shown to be overexpressed in most classical medulloblastomas (Lee et al., 2002). |
Disease | Medulloblastomas are brain tumors occurred in the cerebellum. |
Entity | Haematological malignancies |
Note | Overexpression of SOX11 was first described as a unique marker for a specific malignant B cell lymphoma, mantle cell lymphoma (Ek et al., 2008; Wang et al., 2008). Subsequent studies have demonstrated that Sox11 is also present in: - 50% of hairy cell leukemia (Chen et al., 2009; Dictor et al., 2009); - 25-50% of Burkitt lymphoma cases (Dictor et al., 2009; Mozos et al., 2009); - Almost all of B-cell lymphoblastic leukemia/lymphomas and T-cell lymphoblastic leukemia/lymphomas; - 2/3 cases of T-cell prolymphocytic leukemia (Dictor et al., 2009; Mozos et al., 2009). |
Prognosis | Expression of SOX11 in mantle cell lymphoma is not only a new diagnostic marker but may also carry information related to the clinical and biological behaviour (Wang et al., 2008). |
Entity | Ovarian cancer |
Note | SOX11 is overexpressed in many epithelial ovarian cancers, and loss of Sox11 is associated with a decreased recurrence-free survival and a more aggressive phenotype (Brennan et al., 2009). |
Prognosis | SOX11 is differently expressed in epithelial ovarian cancer and is a prognostic marker. |
Bibliography |
The establishment of neuronal properties is controlled by Sox4 and Sox11. |
Bergsland M, Werme M, Malewicz M, Perlmann T, Muhr J. |
Genes Dev. 2006 Dec 15;20(24):3475-86. |
PMID 17182872 |
The transcription factor Sox11 is a prognostic factor for improved recurrence-free survival in epithelial ovarian cancer. |
Brennan DJ, Ek S, Doyle E, Drew T, Foley M, Flannelly G, O'Connor DP, Gallagher WM, Kilpinen S, Kallioniemi OP, Jirstrom K, O'Herlihy C, Borrebaeck CA. |
Eur J Cancer. 2009 May;45(8):1510-7. Epub 2009 Mar 9. |
PMID 19272768 |
Nuclear expression of sox11 is highly associated with mantle cell lymphoma but is independent of t(11;14)(q13;q32) in non-mantle cell B-cell neoplasms. |
Chen YH, Gao J, Fan G, Peterson LC. |
Mod Pathol. 2010 Jan;23(1):105-12. Epub 2009 Oct 2. |
PMID 19801969 |
Strong lymphoid nuclear expression of SOX11 transcription factor defines lymphoblastic neoplasms, mantle cell lymphoma and Burkitt's lymphoma. |
Dictor M, Ek S, Sundberg M, Warenholt J, Gyorgy C, Sernbo S, Gustavsson E, Abu-Alsoud W, Wadstrom T, Borrebaeck C. |
Haematologica. 2009 Nov;94(11):1563-8. |
PMID 19880779 |
The three SoxC proteins--Sox4, Sox11 and Sox12--exhibit overlapping expression patterns and molecular properties. |
Dy P, Penzo-Mendez A, Wang H, Pedraza CE, Macklin WB, Lefebvre V. |
Nucleic Acids Res. 2008 May;36(9):3101-17. Epub 2008 Apr 10. |
PMID 18403418 |
Nuclear expression of the non B-cell lineage Sox11 transcription factor identifies mantle cell lymphoma. |
Ek S, Dictor M, Jerkeman M, Jirstrom K, Borrebaeck CA. |
Blood. 2008 Jan 15;111(2):800-5. Epub 2007 Oct 12. |
PMID 17934069 |
Expression of Sox11 in adult neurogenic niches suggests a stage-specific role in adult neurogenesis. |
Haslinger A, Schwarz TJ, Covic M, Chichung Lie D. |
Eur J Neurosci. 2009 Jun;29(11):2103-14. Epub 2009 May 21. |
PMID 19490090 |
Sox11 prevents tumorigenesis of glioma-initiating cells by inducing neuronal differentiation. |
Hide T, Takezaki T, Nakatani Y, Nakamura H, Kuratsu J, Kondo T. |
Cancer Res. 2009 Oct 15;69(20):7953-9. Epub 2009 Oct 6. |
PMID 19808959 |
Sox11 transcription factor modulates peripheral nerve regeneration in adult mice. |
Jankowski MP, McIlwrath SL, Jing X, Cornuet PK, Salerno KM, Koerber HR, Albers KM. |
Brain Res. 2009 Feb 23;1256:43-54. Epub 2008 Dec 24. |
PMID 19133245 |
The human SOX11 gene: cloning, chromosomal assignment and tissue expression. |
Jay P, Goze C, Marsollier C, Taviaux S, Hardelin JP, Koopman P, Berta P. |
Genomics. 1995 Sep 20;29(2):541-5. |
PMID 8666406 |
Differential expression of SOX4 and SOX11 in medulloblastoma. |
Lee CJ, Appleby VJ, Orme AT, Chan WI, Scotting PJ. |
J Neurooncol. 2002 May;57(3):201-14. |
PMID 12125983 |
SOX11 expression is highly specific for mantle cell lymphoma and identifies the cyclin D1-negative subtype. |
Mozos A, Royo C, Hartmann E, De Jong D, Baro C, Valera A, Fu K, Weisenburger DD, Delabie J, Chuang SS, Jaffe ES, Ruiz-Marcellan C, Dave S, Rimsza L, Braziel R, Gascoyne RD, Sole F, Lopez-Guillermo A, Colomer D, Staudt LM, Rosenwald A, Ott G, Jares P, Campo E. |
Haematologica. 2009 Nov;94(11):1555-62. |
PMID 19880778 |
Critical roles for SoxC transcription factors in development and cancer. |
Penzo-Mendez AI. |
Int J Biochem Cell Biol. 2009 Aug 3. [Epub ahead of print] |
PMID 19651233 |
Identification of a naturally processed T cell epitope derived from the glioma-associated protein SOX11. |
Schmitz M, Wehner R, Stevanovic S, Kiessling A, Rieger MA, Temme A, Bachmann M, Rieber EP, Weigle B. |
Cancer Lett. 2007 Jan 8;245(1-2):331-6. Epub 2006 Feb 28. |
PMID 16504379 |
Gene targeting reveals a widespread role for the high-mobility-group transcription factor Sox11 in tissue remodeling. |
Sock E, Rettig SD, Enderich J, Bosl MR, Tamm ER, Wegner M. |
Mol Cell Biol. 2004 Aug;24(15):6635-44. |
PMID 15254231 |
Members of the SRY family regulate the human LINE retrotransposons. |
Tchenio T, Casella JF, Heidmann T. |
Nucleic Acids Res. 2000 Jan 15;28(2):411-5. |
PMID 10606637 |
The subcellular Sox11 distribution pattern identifies subsets of mantle cell lymphoma: correlation to overall survival. |
Wang X, Asplund AC, Porwit A, Flygare J, Smith CI, Christensson B, Sander B. |
Br J Haematol. 2008 Oct;143(2):248-52. Epub 2008 Aug 20. |
PMID 18729857 |
Highly specific overexpression of the transcription factor SOX11 in human malignant gliomas. |
Weigle B, Ebner R, Temme A, Schwind S, Schmitz M, Kiessling A, Rieger MA, Schackert G, Schackert HK, Rieber EP. |
Oncol Rep. 2005 Jan;13(1):139-44. |
PMID 15583815 |
Citation |
This paper should be referenced as such : |
Wang, X ; Sander, B |
SOX11 (SRY (sex determining region Y)-box 11) |
Atlas Genet Cytogenet Oncol Haematol. 2010;14(11):1039-1041. |
Free journal version : [ pdf ] [ DOI ] |
External links |
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