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Gene Review

ZNF274  -  zinc finger protein 274

Homo sapiens

Synonyms: HFB101, Neurotrophin receptor-interacting factor homolog, SP2114, ZF2, ZKSCAN19, ...
 
 
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Disease relevance of ZNF274

  • The Evi-1 transcriptional repressor protein has two distinct zinc finger DNA binding domains designated ZF1 and ZF2 and is implicated in the progression of human and murine leukemias, in which it is abnormally expressed [1].
 

High impact information on ZNF274

  • In each case a constitutional point mutation was detected in either ZF2 or ZF3 [2].
  • Whereas ZF2 contains the transactivation function, zinc regulation is dependent on the presence of ZF1 [3].
  • Kinetic lability in Zn(II) binding was mapped to the alpha-helix of ZF2 [3].
  • The data illustrate the involvement of overlapping, but distinct, domains of AKAP95 for condensin recruitment and chromosome condensation and argue for a key role of ZF1 in chromosome condensation and ZF2 in condensin targeting [4].
  • Optimal repression is DNA binding dependent and is mediated by either ZF1 or a heterologous GAL4 DNA binding domain (GAL4DBD) but is significantly less efficient through the ZF2 binding site [5].
 

Biological context of ZNF274

  • Identification and characterization of human ZNF274 cDNA, which encodes a novel kruppel-type zinc-finger protein having nucleolar targeting ability [6].
  • The ZNF274 gene is mapped distal to marker RP S28 1 in the human chromosome 19qter region, by RH mapping [6].
  • In four out of the five patients, heterozygous germline mutations were found: a novel point mutation in exon 8 (ZF2) at codon 377 altering the wild-type histidine to arginine, and three previously described point mutations in exon 9 (ZF3) in the codons corresponding to amino acids 394Arg and 396Asp [7].

References

  1. Loss of cell cycle control by deregulation of cyclin-dependent kinase 2 kinase activity in Evi-1 transformed fibroblasts. Kilbey, A., Stephens, V., Bartholomew, C. Cell Growth Differ. (1999) [Pubmed]
  2. Evidence that WT1 mutations in Denys-Drash syndrome patients may act in a dominant-negative fashion. Little, M.H., Williamson, K.A., Mannens, M., Kelsey, A., Gosden, C., Hastie, N.D., van Heyningen, V. Hum. Mol. Genet. (1993) [Pubmed]
  3. Zinc metalloregulation of the zinc finger pair domain. Bird, A.J., Swierczek, S., Qiao, W., Eide, D.J., Winge, D.R. J. Biol. Chem. (2006) [Pubmed]
  4. Distinct but overlapping domains of AKAP95 are implicated in chromosome condensation and condensin targeting. Eide, T., Carlson, C., Taskén, K.A., Hirano, T., Taskén, K., Collas, P. EMBO Rep. (2002) [Pubmed]
  5. The Evi-1 proto-oncogene encodes a transcriptional repressor activity associated with transformation. Bartholomew, C., Kilbey, A., Clark, A.M., Walker, M. Oncogene (1997) [Pubmed]
  6. Identification and characterization of human ZNF274 cDNA, which encodes a novel kruppel-type zinc-finger protein having nucleolar targeting ability. Yano, K., Ueki, N., Oda, T., Seki, N., Masuho, Y., Muramatsu, M. Genomics (2000) [Pubmed]
  7. WT1 mutations in patients with Denys-Drash syndrome: a novel mutation in exon 8 and paternal allele origin. Nordenskjöld, A., Friedman, E., Anvret, M. Hum. Genet. (1994) [Pubmed]
 
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