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S G Gong

Publications and source records attributed to S G Gong.

9 recordsLinked to original sources
PubMed2001

Characterization of olfactory nerve abnormalities in Twirler mice.

The sense of smell is perceived by olfactory receptor neurons (ORN) present in the olfactory epithelium located in the posterosuperior aspect of the nasal cavity. The axons of these ORN migrate to the olfactory bulb (OB), forming a nervous layer on the outermost part of the bulb, and finally synapse in glomerular structures in the OB. The ORN are unique in that they are constantly being renewed throughout life. We characterized the defects in the nasal cavity and olfactory nervous supply of Twirler (Tw) mice by histological and immunohistochemical means. Tw homozygotes have previously been shown to present with midfacial abnormalities in the form of clefts of the lip and palate (Lyon, 1958; Gong et al., 2000). We found that in the Tw homozygotes, the OB was abnormally shaped, the skeletal framework underlying the OB was disrupted, and the morphology of the nasal cavity was altered with poorly defined nasal turbinates. Immunohistochemical staining with antibodies that marked nerves in general (PGP 9.5) and mature ORN (omp) in the olfactory epithelium at two different embryonic stages and in newborn mice revealed the stratification of the olfactory epithelium in Tw homozygotes, albeit slightly thinner compared to wildtype. A striking difference in the olfactory epithelium was the lack of differentiation of the ORN in Tw homozygotes and the reduced axonal input to the OB. In Tw homozygotes at 14.5 days of embryonic development, the presence of many mature ORN found randomly in the mesenchyme suggests the loss of olfactory pathfinding cues to the OB. It is believed that the lack of appropriate pathfinding cues observed in the Tw homozygotes was responsible for the OB not having the appropriate trophic effect on the development and maturation of the ORN as had been observed in partially bulbectomized animals. The defects in the Twirler may prove to be a valuable system to analyze problems in olfactory pathfinding and maturation.

Animals↗
PubMed2001

Tumorhead, a Xenopus gene product that inhibits neural differentiation through regulation of proliferation.

Tumorhead (TH) is a novel maternal gene product from Xenopus laevis containing several basic domains and a weak coiled-coil. Overexpression of wild-type TH resulted in increased proliferation of neural plate cells, causing expansion of the neural field followed by neural tube and craniofacial abnormalities. Overexpressed TH protein repressed neural differentiation and neural crest markers, but did not inhibit the neural inducers, pan-neural markers or mesodermal markers. Loss of function by injection of anti-TH antibody inhibited cell proliferation. Our data are consistent with a model in which tumorhead functions in regulating differentiation of the neural tissues but not neural induction or determination through its effect on cell proliferation.

Amino Acid Sequence↗
PubMed2001

Phenotypic and molecular analyses of A/WySn mice.

OBJECTIVE: Since its first description, the A strain of mice have been utilized extensively as models to study the processes involved in clefting of the midfacial region. Of the A substrains, the A/WySn has a spontaneous rate of clefting of the lip of about 20% to 30%. The A/WySn mouse model was utilized in this study to analyze and compare the phenotypic and molecular changes in the midfacial region of embryos with and without cleft. RESULTS: Scanning electron microscopy and skeletal and cartilage preparations of newborn A/WySn pups showed the presence of bilateral and unilateral clefts of the lips and the disruption of the skeletal and cartilaginous components of the mice with clefts of the lip. The expression of the msx1 homeobox gene was analyzed by whole mount in situ hybridization of A/WySn embryos at different stages of midfacial development. The results showed that there was misregulation of the expression of the msx1 gene in embryos with cleft, with a persistence of expression in the distal growing tips of the midfacial processes and in areas that have fused in normal embryos without cleft. CONCLUSIONS: Although the genetic defect in A/WySn mice is not known, a possible candidate gene has been mapped to a corresponding human chromosome carrying retinoic acid receptor alpha, and there exists a possibility that msx1 is in the same genetic pathway affected by the mutation of the gene in A/WySn.

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PubMed2001

Palatal development in Twirler mice.

OBJECTIVE: The development of the secondary palate of Twirler (Tw) mice was characterized, and a quantitative coronal plane evaluation of the width and length of the craniofacial regions of homozygous mutant embryos was conducted. RESULTS AND CONCLUSIONS: The secondary palatal shelves were retarded in growth and the timing of elevation from a vertical to horizontal position. The homozygous Tw embryos, when compared with their wild-type littermates, also had a significantly wider midface. It is not surprising, therefore, that the short palatal shelves in a wide midface resulted in cleftings of the secondary palate observed in all homozygous Tw mice. These findings are consistent with similar studies in humans and other murine models.

Animals↗
PubMed2000

The Twirler mouse, a model for the study of cleft lip and palate.

Twirler (Tw) is a semidominant mutation in the mouse affecting the embryonic development of the midfacial region. Most heterozygous Tw mice, +/-, become obese at adulthood with a concomitant decrease in fertility. Homozygous mice have clefts of the midfacial region and a disrupted nasal cavity. Midfacial clefts included clefts of the palate combined with either unilateral or bilateral clefts of the lip. The clefts of the lip were either complete or incomplete. The palatal shelves in Tw/Tw were very much reduced. Apart from these defects, homozygous Tw looked normal, and were born alive, although they reportedly die within 24 h after birth. It is proposed that the Twirler model can be used to improve understanding of the genetic mechanisms involved in the normal development of the midfacial region.

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PubMed1999

A statistical analysis of the overexpression of the msx2 RNA in Xenopus laevis.

The msx family of genes are important during development, and implicated in the development of various craniofacial structures. Here the frog, Xenopus laevis, was used to perform gain-of-function experiments to obtain further insight into the role of the msx2 gene. mRNAs of wild-type and mutated forms of the msx2 gene were injected into developing Xenopus embryos. Phenotypic changes in these embryos were noted, scored, and subjected to statistical analyses. Overexpression of the wild-type form of the msx2 gene resulted in embryos that were ventralized, i.e. with loss of anterior structures including head and eyes. A surprising finding was the statistically significant difference in phenotypic changes (p < 0.001 when compared to a buffer-injected group) of embryos microinjected with the mRNA of a mutated form of the msx2 gene (without the homeobox region). It is proposed that the msx2 overexpression system can be used as a consistent and reliable bioassay to map and study the functions of the msx2 gene during development, especially of the craniofacial region.

Animals↗
PubMed1999

The role of Xmsx-2 in the anterior-posterior patterning of the mesoderm in Xenopus laevis.

Many molecules are involved in defining mesodermal patterning of the Xenopus embryo. In this paper, evidence is provided that a member of the msx family of genes, the Xmsx-2 gene, is involved in anterior-posterior patterning of the mesoderm. A comparison of its sequence to another previously cloned msx-2 Xenopus homolog, Xhox-7.1' [45] showed that they are closely related. The Xmsx-2 gene is first expressed at midgastrulation predominantly in the dorsal part of the embryo. It showed a complex pattern of spatial expression, consistent with a role in patterning of the anterior-posterior axis. This inference is confirmed by gain-of-function experiments in which overexpressed msx-2 mRNA in developing Xenopus embryos resulted in embryos lacking anterior structures. Analysis of markers in mutant embryos showed that genes involved in ventral-posterior patterning such as Xhox-3, Xwnt-8, and Xvent-1 were upregulated, confirming the posteriorized nature of the embryos. We believe that the Xmsx-2 gene is involved in refining the patterning of the anterior-posterior part of the dorsal mesoderm after the initial signals determining the dorsal or ventral nature of the mesoderm have been specified.

Amino Acid Sequence↗
PubMed1995

Two forms of Xenopus nuclear factor 7 have overlapping spatial but different temporal patterns of expression during development.

Xenopus nuclear factor 7 (xnf7) is a maternal gene product that functions in the determination of the dorsal-ventral body axis. We have cloned two xnf7 cDNAs, xnf7-O and xnf7-B, that have a different temporal pattern of expression. The cDNAs differ by 39 amino acid residues scattered throughout the molecule. Most of the changes were conservative in nature. Using gene specific probes we found that xnf7-O transcripts were abundant in oocytes and decreased until the neurula stage, after which they increased in abundance. Xnf7-B transcripts were in low abundance in oocytes and were expressed at high levels at the neurula stage and in adult brain. Both xnf7-O and xnf7-B transcripts at the neurula stage were localized in the dorsal region of the embryo, including the neural folds and somites. Xnf7 was not expressed in ventralized embryos that lacked dorsal structures, thereby substantiating its dorsal localization in the embryo. The promoter region of the xnf7-O gene does not possess a TATA box but does contain E2F, USF, Sp1-like and AP1 binding sites within the first 421 bp from the transcription initiation site. A 62 bp fragment of the xnf7-O promoter containing the Sp1-like and E2F sites can direct proper spatial expression of a transgene in embryos.

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