HGNC Approved Gene Symbol: ZNF200
Cytogenetic location: 16p13.3 Genomic coordinates (GRCh38) : 16:3,222,325-3,235,158 (from NCBI)
During genomic sequencing and exon trapping in the chromosomal region around the familial Mediterranean fever locus (MEFV; 608107), the French FMF Consortium (1997) identified a novel gene encoding a zinc finger protein. The authors amplified all 5 exons from a human liver cDNA library. The open reading frame encodes a 394-amino acid polypeptide.
Deng et al. (1998) also isolated ZNF200 cDNAs and genomic clones and noted 2 differences in the predicted ZNF200 protein sequence. Northern blot analysis detected 2- and 3.5-kb ZNF200 transcripts in all tissues tested, with strongest expression in testis.
Nishida et al. (2007) stated that ZNF200 contains 5 tandem Kruppel-like C2H2 domains, each of which binds a zinc ion to form a globular domain. Western blot analysis detected endogenous ZNF200 at an apparent molecular mass of 50 kD in human embryonic kidney cells. Epitope-tagged ZNF200 colocalized with G9A (EHMT2; 604599) in most areas of the nucleus as discrete speckles. During metaphase, the 2 proteins colocalized outside of mitotic chromosomes.
By yeast 2-hybrid analysis of a fetal brain cDNA library, Nishida et al. (2007) showed that an N-terminal domain of G9A interacted with ZNF200. Immunoprecipitation analysis confirmed interaction between endogenous G9A and ZNF200 in human embryonic kidney cells. Mutation analysis indicated that at least 2 of the 5 zinc finger domains of ZNF200 were required for its interaction with G9A.
The French FMF Consortium (1997) determined that the ZNF200 gene contains 5 exons and spans 14 kb. Deng et al. (1998) determined that the ZNF200 gene contains at least 6 exons, rather than 5 as reported by the French FMF Consortium (1997), and attributed this discrepancy to alternative splicing.
The French FMF Consortium (1997) mapped the ZNF200 gene to chromosome 16p13, between the OR1F1 (603232) and MEFV genes, with MEFV being the more centromeric. By analysis of clones from the MEFV region, Deng et al. (1998) determined that ZNF200 is located approximately 10 kb telomeric to the MEFV gene on chromosome 16p13.3. They reported that 4 other zinc finger genes are located in the MEFV region, including ZNF205 (603636).
Deng, Z., Centola, M., Chen, X., Sood, R., Vedula, A., Fischel-Ghodsian, N., Kastner, D. L. Identification of two Kruppel-related zinc finger genes (ZNF200 and ZNF210) from human chromosome 16p13.3. Genomics 53: 97-103, 1998. [PubMed: 9787081] [Full Text: https://doi.org/10.1006/geno.1998.5430]
French FMF Consortium. A candidate gene for familial Mediterranean fever. Nature Genet. 17: 25-31, 1997. [PubMed: 9288094] [Full Text: https://doi.org/10.1038/ng0997-25]
Nishida, M., Kato, M., Kato, Y., Sasai, N., Ueda, J., Tachibana, M., Shinkai, Y., Yamaguchi, M. Identification of ZNF200 as a novel binding partner of histone H3 methyltransferase G9a. Genes Cells 12: 877-888, 2007. [PubMed: 17584299] [Full Text: https://doi.org/10.1111/j.1365-2443.2007.01098.x]