Nuclear factor I X deficiency causes brain malformation and severe skeletal defects.


:The transcription factor family of nuclear factor I (NFI) proteins is encoded by four closely related genes: Nfia, Nfib, Nfic, and Nfix. A potential role for NFI proteins in regulating developmental processes has been implicated by their specific expression pattern during embryonic development and by analysis of NFI-deficient mice. It was shown that loss of NFIA results in hydrocephalus and agenesis of the corpus callosum and that NFIB deficiency leads to neurological defects and to severe lung hypoplasia, whereas Nfic knockout mice exhibit specific tooth defects. Here we report the knockout analysis of the fourth and last member of this gene family, Nfix. Loss of NFIX is postnatally lethal and leads to hydrocephalus and to a partial agenesis of the corpus callosum. Furthermore, NFIX-deficient mice develop a deformation of the spine, which is due to a delay in ossification of vertebral bodies and a progressive degeneration of intervertebral disks. Impaired endochondral ossification and decreased mineralization were also observed in femoral sections of Nfix-/- mice. Consistent with the defects in bone ossification we could show that the expression level of tetranectin, a plasminogen-binding protein involved in mineralization, is specifically downregulated in bones of NFIX-deficient mice.


Mol Cell Biol


Driller K,Pagenstecher A,Uhl M,Omran H,Berlis A,Gründer A,Sippel AE




Has Abstract


2007-05-01 00:00:00














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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Keskin N,Deniz E,Eryilmaz J,Un M,Batur T,Ersahin T,Cetin Atalay R,Sakaguchi S,Ellmeier W,Erman B

    更新日期:2015-05-01 00:00:00

  • Empirical equation that can be used to determine genetic map distances from tetrad data.

    abstract::An empirical equation has been developed that can be used to calculate genetic map distances from tetrad data with good accuracy for distances of up to at least 120 centimorgans. ...

    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Ma C,Mortimer RK

    更新日期:1983-10-01 00:00:00

  • The histidyl-tRNA synthetase-related sequence in the eIF-2 alpha protein kinase GCN2 interacts with tRNA and is required for activation in response to starvation for different amino acids.

    abstract::Protein kinase GCN2 is a multidomain protein that contains a region homologous to histidyl-tRNA synthetases juxtaposed to the kinase catalytic moiety. Previous studies have shown that in response to histidine starvation, GCN2 phosphorylates eukaryotic initiation factor 2 (eIF-2), to induce the translational expression...

    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Wek SA,Zhu S,Wek RC

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  • Topological requirements for homologous recombination among DNA molecules transfected into mammalian cells.

    abstract::Cultured animal cells rearrange foreign DNA very efficiently by homologous recombination. The individual steps that constitute the mechanism(s) of homologous recombination in transfected DNA are as yet undefined. In this study, we examined the topological requirements by using the genome of simian virus 40 (SV40) as a...

    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Wake CT,Vernaleone F,Wilson JH

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  • Binding of the termination factor Nsi1 to its cognate DNA site is sufficient to terminate RNA polymerase I transcription in vitro and to induce termination in vivo.

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Merkl P,Perez-Fernandez J,Pilsl M,Reiter A,Williams L,Gerber J,Böhm M,Deutzmann R,Griesenbeck J,Milkereit P,Tschochner H

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Ostler N,Britzen-Laurent N,Liebl A,Naschberger E,Lochnit G,Ostler M,Forster F,Kunzelmann P,Ince S,Supper V,Praefcke GJ,Schubert DW,Stockinger H,Herrmann C,Stürzl M

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Widmann C,Gerwins P,Johnson NL,Jarpe MB,Johnson GL

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    pub_type: 杂志文章


    authors: Cowan NJ,Dobner PR,Fuchs EV,Cleveland DW

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    journal_title:Molecular and cellular biology

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    authors: Baba K,Takeshita A,Majima K,Ueda R,Kondo S,Juni N,Yamamoto D

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  • Composite patterns in neutral/neutral two-dimensional gels demonstrate inefficient replication origin usage.

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    journal_title:Molecular and cellular biology

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    authors: Kalejta RF,Hamlin JL

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    journal_title:Molecular and cellular biology

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    authors: Li X,Coffino P

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  • TRF1 controls telomere length and mitotic fidelity in epithelial homeostasis.

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Muñoz P,Blanco R,de Carcer G,Schoeftner S,Benetti R,Flores JM,Malumbres M,Blasco MA

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Wormington WM

    更新日期:1989-12-01 00:00:00

  • Cotranscriptional splicing potentiates the mRNA production from a subset of estradiol-stimulated genes.

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    journal_title:Molecular and cellular biology

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    authors: Bittencourt D,Dutertre M,Sanchez G,Barbier J,Gratadou L,Auboeuf D

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Kass S,Sollner-Webb B

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  • A regulatory role for microRNA 33* in controlling lipid metabolism gene expression.

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    authors: Goedeke L,Vales-Lara FM,Fenstermaker M,Cirera-Salinas D,Chamorro-Jorganes A,Ramírez CM,Mattison JA,de Cabo R,Suárez Y,Fernández-Hernando C

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Tzameli I,Pissios P,Schuetz EG,Moore DD

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  • Fibronectin is overproduced by keloid fibroblasts during abnormal wound healing.

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Babu M,Diegelmann R,Oliver N

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  • HDAC4, a human histone deacetylase related to yeast HDA1, is a transcriptional corepressor.

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


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  • Requirement for a functional interaction between mediator components Med6 and Srb4 in RNA polymerase II transcription.

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    journal_title:Molecular and cellular biology

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    journal_title:Molecular and cellular biology

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Wides RJ,Challberg MD,Rawlins DR,Kelly TJ

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  • Transforming c-Ki-ras mutation is a preneoplastic event in mouse mammary carcinogenesis induced in vitro by N-methyl-N-nitrosourea.

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Miyamoto S,Sukumar S,Guzman RC,Osborn RC,Nandi S

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  • A novel protein factor is required for use of distal alternative 5' splice sites in vitro.

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Harper JE,Manley JL

    更新日期:1991-12-01 00:00:00

  • Functional expression and RNA binding analysis of the interferon-induced, double-stranded RNA-activated, 68,000-Mr protein kinase in a cell-free system.

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Katze MG,Wambach M,Wong ML,Garfinkel M,Meurs E,Chong K,Williams BR,Hovanessian AG,Barber GN

    更新日期:1991-11-01 00:00:00

  • RNA polymerase II carboxy-terminal domain phosphorylation is required for cotranscriptional pre-mRNA splicing and 3'-end formation.

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Bird G,Zorio DA,Bentley DL

    更新日期:2004-10-01 00:00:00

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Mead J,Zhong H,Acton TB,Vershon AK

    更新日期:1996-05-01 00:00:00

  • Induction of hTERT expression and telomerase activity by estrogens in human ovary epithelium cells.

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Misiti S,Nanni S,Fontemaggi G,Cong YS,Wen J,Hirte HW,Piaggio G,Sacchi A,Pontecorvi A,Bacchetti S,Farsetti A

    更新日期:2000-06-01 00:00:00

  • Regulation of glycogen synthase kinase 3beta and downstream Wnt signaling by axin.

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    journal_title:Molecular and cellular biology

    pub_type: 杂志文章


    authors: Hedgepeth CM,Deardorff MA,Rankin K,Klein PS

    更新日期:1999-10-01 00:00:00