Integration of a transposon into the <i>Gli3</i> gene in the <i>Pdn</i> mouse

Ueta, Etsuko; Nanba, Eiji

doi:10.1111/j.1741-4520.2002.tb00898.x

Cited by 15 publications

(31 citation statements)

References 28 publications

(30 reference statements)

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“…This analysis also revealed low amounts of an additional Pdn/Pdn-specific Gli3 protein of ϳ85 kDa (Fig. 1 I, asterisk), which is likely to be encoded by previously described alternative splice products, leading to the insertion of 56 or 61 aa in the N-terminal part of the Gli3 protein (Thien and Rüther, 1999;Ueta et al, 2002). The presence of a Gli3 activator protein containing the same additional amino acids is likely, but could not be resolved on these gels due to its high molecular weight (170 kDa compared with 175 kDa).…”

Section: Resultsmentioning

confidence: 99%

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TheGli3Hypomorphic MutationPdnCauses Selective Impairment in the Growth, Patterning, and Axon Guidance Capability of the Lateral Ganglionic Eminence

Magnani¹,

Hasenpusch‐Theil²,

Jacobs³

et al. 2010

J. Neurosci.

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Previous studies have defined a requirement for Sonic hedgehog (Shh) signaling in patterning the ventral telencephalon, a major source of the neuronal diversity found in the mature telencephalon. The zinc finger transcription factor Gli3 is a critical component of the Shh signaling pathway and its loss causes major defects in telencephalic development. Gli3 is expressed in a graded manner along the dorsoventral axis of the telencephalon but it is unknown whether Gli3 expression levels are important for dorsoventral telencephalic patterning. To address this, we used the Gli3 hypomorphic mouse mutant Polydactyly Nagoya (Pdn). We show that in Pdn/Pdn embryos, the telencephalic expression of Gli3 remains graded, but Gli3 mRNA and protein levels are reduced, resulting in an upregulation of Shh expression and signaling. These changes mainly affect the development of the lateral ganglionic eminence (LGE), with some disorganization of the medial ganglionic eminence mantle zone. The pallial/subpallial boundary is shifted dorsally and the production of postmitotic neurons is reduced. Moreover, LGE pioneer neurons that guide corticofugal axons into the LGE do not form properly, delaying the entry of corticofugal axons into the ventral telencephalon. Pdn/Pdn mutants also show severe pathfinding defects of thalamocortical axons in the ventral telencephalon. Transplantation experiments demonstrate that the intrinsic ability of the Pdn ventral telencephalon to guide thalamocortical axons is compromised. We conclude that correct Gli3 levels are particularly important for the LGE's growth, patterning, and development of axon guidance capabilities.

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Section: Resultsmentioning

confidence: 99%

“…Embryonic (E) day 0.5 was assumed to start at midday of the day of vaginal plug discovery. Embryos were genotyped as described previously (Ueta et al, 2002). In qualitative analyses of mutant phenotypes, heterozygous and wildtype embryos did not show differences and both were used as control embryos.…”

Section: Methodsmentioning

confidence: 99%

TheGli3Hypomorphic MutationPdnCauses Selective Impairment in the Growth, Patterning, and Axon Guidance Capability of the Lateral Ganglionic Eminence

Magnani¹,

Hasenpusch‐Theil²,

Jacobs³

et al. 2010

J. Neurosci.

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“…Gli3 is the responsible gene for the Pdn mouse (Thien & Rüther 1999; Naruse et al . 2000, 2001; Ueta et al . 2002) and GLI3 for GCPS (Vortkamp et al .…”

Section: Introductionmentioning

confidence: 99%

Genetic susceptibility in the neural tube defects induced by ochratoxin A in the genetic arhinencephaly mouse,Pdn/Pdn

Ohta

Maekawa

Katagiri

et al. 2006

Congenital Anomalies

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It is well known that ochratoxin A (OTA) induces neural tube defects (NTDs) in mice. In the present study, OTA was administered to the genetic polydactyly/arhinencephaly mouse (Pdn/Pdn) to investigate the synergistic effect between gene and environmental toxin. OTA treatment on day 7.5 of gestation increased NTDs in the Pdn/Pdn mouse. The responsible gene for Pdn/Pdn is Gli3. So, it was speculated that specific susceptibility for OTA in the Pdn/Pdn mouse embryo may be due to the severe depression of Gli3 gene expression. As correlated genes, Gli3, Shh and Fgf8 gene expressions were examined in the Pdn mouse embryo on day 9 of gestation after administration of OTA on day 7.5. No alteration of Shh expression was observed in the non-treated Pdn/Pdn, and OTA-treated +/+ and Pdn/Pdn. Fgf8 signal was observed at the anterior neural ridge (ANR) in the non-treated +/+, and that was elongated in the non-treated Pdn/Pdn, and further elongated and more intensive in the OTA-treated Pdn/Pdn. It was suggested that Fgf8 gene expression was affected by the depression of Gli3, and alteration of Fgf8 gene expression was accelerated by the toxicity of OTA in the Pdn/Pdn.

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“…Therefore, Pdn/Pdn mice do not seem to have any thumbs/big toes because neither factor is found in the fore/hindlimbs of Pdn/Pdn mice. In the molecular study, Ueta et al (2002) observed the suppression of Gli3 gene expression in Pdn mice and considered Gli3 to be the gene responsible for Pdn/Pdn mutant mice. According to Lallemand et al (2005), Msx1 and Msx2 are highly expressed in early limb formation, and anterior skeletal elements, such as thumb/hallux and radius/tibia, are lacking in Msx1 and Msx2 doublemutant mice.…”

Section: Discussionmentioning

confidence: 99%

Normal Location Of Thumb/Big Toe may be Related to Programmed Cell Death in the Preaxial Area of Embryonic Limb

Kimura

Yamada

2011

The Anatomical Record

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The forelimbs and hindlimbs of newborn Polydactyly Nagoya (Pdn) mice were examined to analyze the roles of programmed cell death (PCD) in the preaxial region of the limb. Special attention was paid to the relationship between the PCD in the preaxial area and the location and shape of the first digit (thumb/big toe). Although a large, bifurcated or duplicated thumb/big toe appeared in Pdn/þ mice, digit I (thumb/big toe) in Pdn/þ mice, as in þ/þ ones, was located more ventro-proximally than the other four digits. On the other hand, abnormal preaxial digits of the fore/hindlimb in Pdn/Pdn mice lay distally and were aligned at the radial/tibial end of a serial curved plane formed by digits II-V; that is, a thumb and big toe of normal shape and location were not detectable in any preaxial digits of Pdn/Pdn mice. In the limb development of Pdn mouse embryos on Day 11-12, PCD did not occur in the preaxial mesoderm of fore/hindlimb only in one-fourths of all embryos obtained by Pdn/þ x Pdn/þ mating. In addition to digital rays II-V, extra preaxial digital rays appeared in the prominent preaxial expansion of fore/hindlimbs in these embryos on early Day 12. These abnormal limb configurations in embryos were closely similar to those in Pdn/Pdn newborn mice. The present findings suggest that PCD in the preaxial region not only prevents the formation of extra digits but also determines the location of the thumb/big toe for the normal limb morphogenesis. Anat Rec, 294:1352Rec, 294: -1359Rec, 294: , 2011. V V C 2011 Wiley-Liss, Inc.

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Integration of a transposon into the Gli3 gene in the Pdn mouse

Cited by 15 publications

References 28 publications

TheGli3Hypomorphic MutationPdnCauses Selective Impairment in the Growth, Patterning, and Axon Guidance Capability of the Lateral Ganglionic Eminence

TheGli3Hypomorphic MutationPdnCauses Selective Impairment in the Growth, Patterning, and Axon Guidance Capability of the Lateral Ganglionic Eminence

Genetic susceptibility in the neural tube defects induced by ochratoxin A in the genetic arhinencephaly mouse,Pdn/Pdn

Normal Location Of Thumb/Big Toe may be Related to Programmed Cell Death in the Preaxial Area of Embryonic Limb

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