Expression of the helix-loop-helix genes Id-1 and NSCL-1 during cerebellar development

Duncan, Melinda K.; Bordas, Lajos; DiCicco‐Bloom, Emanuel; Chada, Kiran

doi:10.1002/(sici)1097-0177(199701)208:1<107::aid-aja10>3.0.co;2-3

Cited by 32 publications

(15 citation statements)

References 43 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…G0/S2 was down-regulated in the homozygous and heterozygous Foxp2 (R552H)-KI brain, suggesting the delay of the cerebral development in the homozygous and heterozygous Foxp2 (R552H)-KI brain. Down-regulation of Nhlh1, which encodes a helix-loop-helix transcriptional protein involved in the differentiation of neural cells and cerebellar development (23), also suggests the immature development of the neuronal cells in the homozygous and heterozygous Foxp2 (R552H) cerebellum. At present, the biological significance of up-regulation of Tac1 is not known, but it may be worth noting that Tac1 is a precursor of substance P expressed in area X of songbirds' brains, in which Foxp2 is up-regulated during vocal learning (11).…”

Section: Discussion Common Molecular Mechanism Shared With Mouse Usvsmentioning

confidence: 99%

Ultrasonic vocalization impairment of Foxp2 (R552H) knockin mice related to speech-language disorder and abnormality of Purkinje cells

Fujita

Tanabe²,

Shiota³

et al. 2008

Proc. Natl. Acad. Sci. U.S.A.

200

205

View full text Add to dashboard Cite

Previous studies have demonstrated that mutation in the forkhead domain of the forkhead box P2 (FOXP2) protein (R553H) causes speech-language disorders. To further analyze FOXP2 function in speech learning, we generated a knockin (KI) mouse for Foxp2 (R552H) [Foxp2 (R552H)-KI], corresponding to the human FOXP2 (R553H) mutation, by homologous recombination. Homozygous Foxp2 (R552H)-KI mice showed reduced weight, immature development of the cerebellum with incompletely folded folia, Purkinje cells with poor dendritic arbors and less synaptophysin immunoreactivity, and achieved crisis stage for survival 3 weeks after birth. At postnatal day 10, these mice also showed severe ultrasonic vocalization (USV) and motor impairment, whereas the heterozygous Foxp2 (R552H)-KI mice exhibited modest impairments. Similar to the wild-type protein, Foxp2 (R552H) localized in the nuclei of the Purkinje cells and the thalamus, striatum, cortex, and hippocampus (CA1) neurons of the homozygous Foxp2 (R552H)-KI mice (postnatal day 10), and some of the neurons showed nuclear aggregates of Foxp2 (R552H). In addition to the immature development of the cerebellum, Foxp2 (R552H) nuclear aggregates may further compromise the function of the Purkinje cells and cerebral neurons of the homozygous mice, resulting in their death. In contrast, heterozygous Foxp2 (R552H)-KI mice, which showed modest impairment of USVs with different USV qualities and which did not exhibit nuclear aggregates, should provide insights into the common molecular mechanisms between the mouse USV and human speech learning and the relationship between the USV and motor neural systems.KE family ͉ nuclear aggregation ͉ autism ͉ endoplasmic reticulum stress T he phenotype of the speech-language disorder segregates as an autosomal dominant trait. The KE family consists of three generations in which approximately half of the members (15 members) have severe articulation difficulties accompanied by verbal and orofacial impairments. The speech difficulties cannot be fully attributed to the basic impairment of orofacial praxis; the affected KE members normally perform single simple oral movements but have trouble with language comprehension, including grammar as well as production (1-5). Recent studies in the KE family identified the forkhead box P2 (FOXP2) gene as the responsible genetic factor and found that a missense mutation (R553H) in the forkhead domain of FOXP2 cosegregates with the disorder in this family (2-5).In addition to a forkhead domain with a winged-helix DNA binding domain (6), FOXP2 also contains a glutamine-rich region (polyQ tract), zinc finger, and a leucine zipper motif for homodimerization and heterodimerization with Foxp1, 2, and 4 family members (7). FOXP2 also interacts with the C-terminal binding protein (CtBP) to act as a transcriptional repressor (7). The familial FOXP2 mutated protein (R553H) exhibits reduced DNA binding and defects in nuclear localization in vitro (8, 9). In addition, a nonsense mutation (R328X), another mutation related to the spe...

show abstract

Section: Discussion Common Molecular Mechanism Shared With Mouse Usvsmentioning

confidence: 99%

Ultrasonic vocalization impairment of Foxp2 (R552H) knockin mice related to speech-language disorder and abnormality of Purkinje cells

Fujita

Tanabe²,

Shiota³

et al. 2008

Proc. Natl. Acad. Sci. U.S.A.

200

205

View full text Add to dashboard Cite

show abstract

“…The overexpression of Wnt-1 directs P19 cells to differentiate into neurons, but not astrocytes (25), perhaps via the enhancement of Ngn-1 gene expression (52). On the other hand, the Id-1 gene encodes an HLH factor to form a heterodimer with bHLH factors and inhibits the DNA-binding function of bHLH factors (53,54). Consequently, the reduced expression of Id-1 may activate the neurogenic bHLH factors (including Mash-1) to aid in the neuronal differentiation of P19 cells.…”

Section: Discussionmentioning

confidence: 99%

Involvement of the Mouse Prp19 Gene in Neuronal/Astroglial Cell Fate Decisions

Urano

Iiduka

Sugiyama

et al. 2006

Journal of Biological Chemistry

View full text Add to dashboard Cite

“…Many groups have reported the detailed spatio-temporal expression patterns of the respective Id genes (Duncan et al, 1992(Duncan et al, , 1997Neuman et al, 1993;Riechmann and Sablitzky, 1995;Zhu et al, 1995;Jen et al, 1996Jen et al, , 1997Tzeng and de Vellis, 1998;Andres-Barquin et al, 2000). In general, all of the factors are expressed in developing neuroblasts, with similar patterns for Id1, Id2 and Id3, and a distinct pattern for Id4, as in the developing non-neural tissues.…”

Section: The Nervous Systemmentioning

confidence: 99%

Id and development

2001

View full text Add to dashboard Cite

During development, it is obvious that enormous multiplication and diversi®cation of cells is required to build a body plan from a single fertilized egg and that these two processes, proliferation and di erentiation, must be coordinated properly. Id proteins, negative regulators of basic helix ± loop ± helix transcription factors, possess the ability to inhibit di erentiation and to stimulate proliferation, and are useful molecules for investigating the mechanisms regulating development. In the past few years, our understanding of the roles of Id proteins has been substantially enhanced by the detailed investigation of genetically modi®ed animals. The data have indicated that the functions of Id proteins in vivo are functionally related to those revealed by earlier work in cell culture systems. However, unexpected organs and cell types have also been found to require Id proteins for their normal development. This review looks at the advances made in our understanding of the in vivo functions of Id proteins. The topics discussed include neurogenesis, natural killer cell development, lymphoid organogenesis, mammary gland development and spermatogenesis. Oncogene (2001) 20, 8290 ± 8298

show abstract

Expression of the helix-loop-helix genes Id-1 and NSCL-1 during cerebellar development

Cited by 32 publications

References 43 publications

Ultrasonic vocalization impairment of Foxp2 (R552H) knockin mice related to speech-language disorder and abnormality of Purkinje cells

Ultrasonic vocalization impairment of Foxp2 (R552H) knockin mice related to speech-language disorder and abnormality of Purkinje cells

Involvement of the Mouse Prp19 Gene in Neuronal/Astroglial Cell Fate Decisions

Id and development

Contact Info

Product

Resources

About