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Gene Review:

ZNF41 - zinc finger protein 41

Homo sapiens

Synonyms: MGC8941, MRX89, Zinc finger protein 41

Rosati, M. et al., Shoichet, S.A. et al., Knight, J.C. et al., Franzè, A. et al., Lugtenberg, D. et al.

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Disease relevance of ZNF41

Mutations in the ZNF41 gene are associated with cognitive deficits: identification of a new candidate for X-linked mental retardation [1].

Psychiatry related information on ZNF41

In silico sequence analysis provided no indication of a causative role for the chromosome 7 breakpoint in mental retardation (MR), whereas, on the X chromosome, a zinc-finger gene, ZNF41, was found to be disrupted [1].

High impact information on ZNF41

ZNF674 belongs to a cluster of seven highly related zinc-finger genes in Xp11, two of which (ZNF41 and ZNF81) were implicated previously in XLMR [2].
Expression studies indicated that ZNF41 transcripts are absent in the patient cell line, suggesting that the mental disorder in this patient results from loss of functional ZNF41 [1].
Wild-type ZNF41 contains a highly conserved transcriptional repressor domain that is linked to mechanisms of chromatin remodeling, a process that is defective in various other forms of MR [1].
The ZNF21, ZNF41, and ZNF81 genes encode Krüppel-type zinc-finger proteins (ZFPs) and have previously been mapped to chromosome Xp [3].
Nucleotide sequence analysis reveals that ZNF41 potentially encodes a polypeptide featuring an array of 18 contiguous zinc fingers of the C2H2 type [4].

Biological context of ZNF41

The expression pattern in cell hybrids containing either active or inactive X chromosomes indicates that ZNF41, which resides within a region of the X chromosome that includes genes that are both subject to and escape X-inactivation, is susceptible to X-chromosome inactivation [5].
Since the KRAB/FPB-A and KRAB/FPB-B modules are encoded by dedicated exons in ZNF41 and paralogous genes, exon skipping may lead to differential usage of these modules in alternative gene products [5].
Coding region intron/exon organization, alternative splicing, and X-chromosome inactivation of the KRAB/FPB-domain-containing human zinc finger gene ZNF41 [5].

Other interactions of ZNF41

ZNF21, ZNF41, and ZNF81 were then shown to segregate within a series of YACs (95 to 730 kb) containing known markers at Xp11.23, such that these YACs could be assembled into a contig spanning approximately 1.5 Mb of DNA [3].

Analytical, diagnostic and therapeutic context of ZNF41

RT-PCR analysis of ZNF41 mRNAs showed that, while skipping of the KRAB/FPB-A and/or KRAB/FPB-B exons was not detected, the use of alternative donor/acceptor sites upstream of the KRAB/FPB-A exon generates multiple ZNF41 transcripts potentially encoding polypeptides differing in the N-terminal region and expressed in different tissues [5].

References

Mutations in the ZNF41 gene are associated with cognitive deficits: identification of a new candidate for X-linked mental retardation. Shoichet, S.A., Hoffmann, K., Menzel, C., Trautmann, U., Moser, B., Hoeltzenbein, M., Echenne, B., Partington, M., Van Bokhoven, H., Moraine, C., Fryns, J.P., Chelly, J., Rott, H.D., Ropers, H.H., Kalscheuer, V.M. Am. J. Hum. Genet. (2003) [Pubmed]
ZNF674: A New Kruppel-Associated Box-Containing Zinc-Finger Gene Involved in Nonsyndromic X-Linked Mental Retardation. Lugtenberg, D., Yntema, H.G., Banning, M.J., Oudakker, A.R., Firth, H.V., Willatt, L., Raynaud, M., Kleefstra, T., Fryns, J.P., Ropers, H.H., Chelly, J., Moraine, C., Gecz, J., Reeuwijk, J., Nabuurs, S.B., de Vries, B.B., Hamel, B.C., de Brouwer, A.P., Bokhoven, H. Am. J. Hum. Genet. (2006) [Pubmed]
Clustered organization of Krüppel zinc-finger genes at Xp11.23, flanking a translocation breakpoint at OATL1: a physical map with locus assignments for ZNF21, ZNF41, ZNF81, and ELK1. Knight, J.C., Grimaldi, G., Thiesen, H.J., Bech-Hansen, N.T., Fletcher, C.D., Coleman, M.P. Genomics (1994) [Pubmed]
Isolation and expression analysis of a human zinc finger gene (ZNF41) located on the short arm of the X chromosome. Franzè, A., Archidiacono, N., Rocchi, M., Marino, M., Grimaldi, G. Genomics (1991) [Pubmed]
Coding region intron/exon organization, alternative splicing, and X-chromosome inactivation of the KRAB/FPB-domain-containing human zinc finger gene ZNF41. Rosati, M., Franzé, A., Matarazzo, M.R., Grimaldi, G. Cytogenet. Cell Genet. (1999) [Pubmed]

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