HGNC Approved Gene Symbol: ZNF133
Cytogenetic location: 20p11.23 Genomic coordinates (GRCh38): 20:18,288,527-18,316,996 (from NCBI)
Transcriptional regulatory proteins containing tandemly repeated zinc finger domains are thought to be involved in both normal and abnormal cellular proliferation and differentiation. One abundant class of such transcriptional regulators resembles the Drosophila Kruppel segmentation gene product due to the presence of repeated Cys2-His2 (C2H2) zinc finger domains that are connected by conserved sequences, called H/C links. See ZNF91 (603971) for general information on zinc finger proteins.
By screening a human insulinoma cDNA library with a degenerate oligonucleotide corresponding to the H/C linker sequence, Tommerup et al. (1993) isolated cDNAs potentially encoding zinc finger proteins. Tommerup and Vissing (1995) performed sequence analysis on a number of these cDNAs and identified several novel zinc finger protein genes, including ZNF133. They found 2 forms of ZNF133 cDNA representing differentially spliced transcripts; one predicts a 654-amino acid (GenBank U09366) Kruppel-type zinc finger protein containing an N-terminal KRAB domain. Vissing et al. (1995) reported that the deduced ZNF133 protein contains 13 zinc finger domains preceeded by a putative degenerate finger domain, and both the KRAB A and B boxes. Northern blot analysis detected ZNF133 expression in all tissues examined.
Margolin et al. (1994) demonstrated that the KRAB domain of ZNF133 functions as a potent transcriptional repressor domain when fused to the yeast GAL4 DNA-binding domain.
By FISH, Tommerup and Vissing (1995) mapped the ZNF133 gene to 20p11.23-p11.22.
Margolin, J. F., Friedman, J. R., Meyer, W. K., Vissing, H., Thiesen, H.-J., Rauscher, F. J., III. Kruppel-associated boxes are potent transcriptional repression domains. Proc. Nat. Acad. Sci. 91: 4509-4513, 1994. [PubMed: 8183939] [Full Text: https://doi.org/10.1073/pnas.91.10.4509]
Tommerup, N., Aagaard, L., Lund, C. L., Boel, E., Baxendale, S., Bates, G. P., Lehrach, H., Vissing, H. A zinc-finger gene ZNF141 mapping at 4p16.3/D4S90 is a candidate gene for the Wolf-Hirschhorn (4p-) syndrome. Hum. Molec. Genet. 2: 1571-1575, 1993. [PubMed: 8268908] [Full Text: https://doi.org/10.1093/hmg/2.10.1571]
Tommerup, N., Vissing, H. Isolation and fine mapping of 16 novel human zinc finger-encoding cDNAs identify putative candidate genes for developmental and malignant disorders. Genomics 27: 259-264, 1995. [PubMed: 7557990] [Full Text: https://doi.org/10.1006/geno.1995.1040]
Vissing, H., Meyer, W. K.-H., Aagaard, L., Tommerup, N., Thiesen, H.-J. Repression of transcriptional activity by heterologous KRAB domains present in zinc finger proteins. FEBS Lett. 369: 153-157, 1995. [PubMed: 7649249] [Full Text: https://doi.org/10.1016/0014-5793(95)00728-r]