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PubMed1995

Cerebellar dermoid tumor and occipital meningocele in a monozygotic twin: clues to the embryogenesis of craniospinal dysraphism.

A case of monochorionic/monoamnionic twin with discordant occipital developmental malformations is presented. One female twin appeared to have an occipital meningocele with cerebellar aplasia and died immediately after birth. The other twin presented with signs and symptoms of raised intracranial pressure at the age of 7 months. Severe hydrocephalus was present due to an infected intracerebellar dermoid tumor with a contiguous occipital dermal sinus. The clinical and pathological characteristics are described and the different theories concerning twinning, embryogenesis, and dysmorphology in relation to neural tube defects are discussed. Analysis of the features of these monozygotic twins indicates that a meningocele is not a post-neurulation disorder but results from deficient neurulation, probably due to mesodermal insufficiency.

Cerebellar Neoplasms↗

PubMed1999

From conception to the child.

An attempt to present all the data that account for the progressive transformation of a fertilized zygote into a child at birth is obviously a herculean work. In this review, I focus on four aspects of central nervous system development in which our understanding has considerably been enhanced during the last century. The first part concerns the phenomenon of neural induction, a process devoted to the acquisition of neural identity. It was classically considered that active molecules were secreted by the organizer (the dorsal lip of the blastopore in amphibians and the node in amniotes) and that these molecules induced the neural induction. Nowadays, the model is much more complex, based upon both positive and negative regulatory mechanisms. The positive inductor is BMP4, and this induces the epidermal induction. The organizer secretes negative signals that can bind BMP4 and prevent its action. The second part is devoted to the morphogenetic movements that allow formation of the neural tube. Classically, primary and secondary neurulations are opposed. It is now well established that the two modes of neurulation obey the same basic rules. The problem of segmentation of the central nervous system during embryogenesis could be easily studied for the rhombencephalon. This structure is formed by repetitive subunits called rhombomeres. Each rhombomere corresponds to a compartment separated from the others. In addition, each rhombomere expresses specific genes, and the invalidation of some of them leads to the absence of these rhombomeres. However, the situation is much more complex with regions specified very early during development and acting on adjacent regions to induce a specific pattern. The last part of my paper concerns the development of the cortex. During the last 50 years, all the classic concepts have been challenged. The fixed law of radial migration has been extensively discussed, and it is now admitted that other kinds of migration do take place during corticogenesis. These results force us to reconsider the interpretations of some cortical malformations. It seems reasonable to adopt descriptive terms for a malformative syndrome instead of terms based on putative mechanisms.

Abnormalities, Multiple↗

PubMed2001

Structure and expression of an Otx5-related gene in the dogfish Scyliorhinus canicula: evidence for a conserved role of Otx5 and Crxgenes in the specification of photoreceptors.

We report the full-length coding sequence and the expression pattern during neurulation and early organogenesis of ScOtx5, a novel member of the Otx gene family in the dogfish Scyliorhinus canicula. Phylogenetic analyses confirm that ScOtx5 is closely related to the Xenopus XlOtx5/ 5bgenes, and also to the Crx genes characterized in mammals and zebrafish. This supports the hypothesis that these genes define a third gnathostome Otx orthology class. During neurulation, ScOtx5 transcripts are detected in the foregut diverticulum and the anterior neuroectoderm. At the onset of organogenesis, ScOtx5 is transcribed over a broad domain spanning the whole prosencephalon and mesencephalon, albeit with a much lower signal intensity than its paralogues Otx1 and Otx2. At later stages, four major expression sites are observed: the developing eye and epiphysis, the olfactory placodes and a broad epidermal domain in the dorsal part of the head. In the embryonic eye, the signal is first detected in the presumptive pigmented retina and slightly later in the adjacent outer layer of the neural retina, fated to photoreceptors. The comparison of this expression pattern with those of osteichthyan Otx genes suggests that a role in the specification of photoreceptors may correspond to a functional specialization of Otx5and Crx genes, fixed early in the gnathostome lineage, prior to the splitting of chondrichthyans and osteichthyans. In contrast, the roles played by ScOtx5 in the retinal pigmented epithelium or in the olfactory placodes may be fulfilled by different combinations of paralogous genes in other gnathostome taxa.

Amino Acid Sequence↗

PubMed2002

Expression patterns of an Otx2 and an Otx5 orthologue in the urodele Pleurodeles waltl: implications on the evolutionary relationships between the balancers and cement gland in amphibians.

We report the characterization of an Otx2 and an Otx5 orthologue in the urodele Pleurodeles waltl. These two genes, termed PwOtx2 and PwOtx5, share highly conserved expression domains with their gnathostome counterparts at tailbud stages, like the developing forebrain ( PwOtx2), or the embryonic eye and epiphysis ( PwOtx5). As in Xenopus laevis, both are also transcribed in the dorsal lip of the blastopore during gastrulation and in anterior parts of the neural plate during neurulation. In addition, PwOtx5 displays a prominent expression in the developing balancers and the lateral non-neural ectoderm during neurulation, from which they derive. By contrast, PwOtx2 expression remains undetectable in the balancers and their presumptive territory. These data suggest that PwOtx5, but not PwOtx2, may be involved in the differentiation and early specification of balancers. Comparisons of Otx5 expression patterns in P. waltland X. laevis embryos suggest that, as previously shown for Otx2, changes in the regulatory mechanisms controlling Otx5 early expression in the non-neural ectoderm may occur frequently among amphibians. These changes may be related to the rise of cement glands in anurans and of balancers in urodeles. This hypothesis could account for some similarities between the two organs, but does not support a homology relationship between them.

Animals↗

PubMed2004

Development of the posterior neural tube in human embryos.

Development of the posterior neural tube (PNT) in human embryos is a complicated process that involves both primary and secondary neurulation. Because normal development of the PNT is not fully understood, pathogenesis of spinal neural tube defects remains elusive. To clarify the mechanism of PNT development, we histologically examined 20 human embryos around the stage of posterior neuropore closure and found that the developing PNT can be divided into three parts: 1) the most rostral region, which corresponds to the posterior part of the primary neural tube, 2) the junctional region of the primary and secondary neural tubes, and 3) the caudal region, which emerges from the neural cord. In the junctional region, the axially-condensed mesenchyme (AM) intervened between the neural plate/tube and the notochord at the stage of posterior neuropore closure, while the notochord was directly attached to the neural plate/tube in the most rostral region. A single cavity was found to be formed in the AM as the presumptive luminal surface cells were radially aligned in the junctional region prior to the formation of the neural cord. The single cavity was continuous with the central cavity of the primary neural tube. In contrast, multiple or isolated cavities were frequently observed in the caudal region of the PNT. Our observation suggests that the junctional region of the PNT is distinct from other regions in terms of the relationship with the notochord and the mode of cavitation during secondary neurulation.

Cauda Equina↗

PubMed1985

Analysis of metabolites of 2-acetylaminofluorene generated in an embryo culture system. Relationship of biotransformation to teratogenicity in vitro.

2-Acetylaminofluorene (AAF) produced abnormal, open neural tubes in cultured whole rat embryos only in the presence of an added, NADPH-dependent monooxygenase system. Reactive intermediary metabolites, including N-hydroxy-AAF, N-hydroxy-2-aminofluorene, 2-nitrosofluorene and N-acetoxy-AAF, each elicited embryonic malformations under culture conditions, but a statistically significant increase in the incidence of abnormal neurulation was not observed. Using [14C]AAF and high pressure liquid chromatography (HPLC) separation techniques, the biotransformation of AAF was studied under conditions in which embryos and the monooxygenase system were coincubated. The major metabolites produced cochromatographed with 5-hydroxy-AAF, 7-hydroxy-AAF, 9-hydroxy-AAF and 3-hydroxy-AAF. Other metabolic products also were detected. The embryonic effects of these major AAF metabolites were tested singly and in combination in the embryo culture system. Addition of 7-hydroxy-AAF to the embryo culture system resulted in open neural tubes in the absence of an added monooxygenase system. Other individual ring-hydroxylated metabolites produced retarded growth, but neurulation appeared normal. Ring-hydroxylated metabolites, added to the embryo culture system in combination in the same proportions as were formed during biotransformation in culture, also produced a marked increase in incidence of neural tube defects in the absence of an exogenous (added) biotransforming system. In combination with 3-, 5- and 9-hydroxy-AAF, 7-hydroxy-AAF exposure (86 microM) resulted in a 47% incidence of abnormal, open neural tubes. When tested individually, higher concentrations of 7-hydroxy-AAF (104 microM) produced a lower percentage of malformed embryos (13%). The results suggested that 7-hydroxy-AAF was principally responsible for the neural tube defects caused by AAF following monooxygenase-dependent bioactivation, but that other metabolites also appeared to contribute to the observed effect.

2-Acetylaminofluorene↗

PubMed1989

Influence of electrophilic character and glutathione depletion on chemical dysmorphogenesis in cultured rat embryos.

To examine the importance of reduced intracellular glutathione (GSH) in the modulation of dysmorphogenesis and to gain insight into the electrophilic character of the embryotoxic intermediates generated in the rat embryo from N-acetoxy-2-acetylaminofluorene (AAAF) and acetaminophen (APAP) in cultured embryos, the effects of GSH depletion on the embryotoxicity, dysmorphogenesis and covalent binding of these agents were examined. Both AAAF (90 microM) and APAP (500 microM) produced concentration-dependent, statistically significant (P less than or equal to 0.05) decreases in embryonic length as well as embryonic and visceral yolk sac protein content when rat embryos were exposed in vitro between days 10 and 11 of gestation. The predominant malformations observed upon exposure to AAAF and APAP were prosencephalic hypoplasia and abnormal neurulation respectively. Exposure of conceptuses to [3H]APAP followed by separation and fractionation of the cellular RNA, DNA and protein via density gradient centrifugation resulted in detectable binding in fractions that contained protein, but not DNA or RNA. This suggested that the rat conceptus is capable of bioactivating APAP to a soft electrophile that selectively arylates protein. In contrast, conceptuses exposed to [3H]AAAF exhibited detectable binding to RNA, DNA and protein, indicative of conversion to both hard and soft electrophiles. Depletion of GSH was accomplished by pretreating conceptuses with 500 microM L-buthionine-S,R-sulfoximine (BSO) from the start of the culture period (day 9.5) until the morning of day 10. When conceptuses were depleted previously of GSH by BSO, exposure to APAP resulted in significant potentiation (relative to APAP alone) of the observed embryotoxicity. These conceptuses displayed further decreases in both embryonic size and protein content of the embryo and yolk sac, as well as increased incidence of abnormally open anterior neuropores and increased binding (3-fold) of [3H]APAP to protein. In contrast, pretreatment with BSO did not potentiate the AAAF-elicited decreases in embryonic size or protein content, nor the severity of prosencephalic hypoplasia, although a slight increase in binding of [3H]AAAF to DNA was observed. Taken together, these data are consistent with the concept that abnormal neurulation elicited by APAP results from the generation of one or more soft electrophilic species, whereas elicitation of prosencephalic hypoplasia by AAAF appears to be a consequence of conversion to a relatively hard electrophile(s).

2-Acetylaminofluorene↗

PubMed1984

Developmentally regulated plasmalemmal glycoconjugates of the surface and neural ectoderm.

The plasmalemmal glycoconjugates of the ectoderm surrounding the rat embryo's caudal neuropore were mapped at the ultrastructural level, using various lectin probes. These included the agglutinins of wheat germ, soybean, Ricinus communis, Lotus tetragonolobus, and Canavalia ensiformis. Each lectin produced a characteristic binding pattern. Comparison of precursor cells of surface ectoderm, neural crest, and neural tube revealed that, even prior to neural tube formation, these three cell types can be distinguished by the sets of lectin receptors they express on their apical plasmalemma. The high density of lectin receptors found at the open neural groove level decreases dramatically during neurulation. Further changes in surface glycoconjugates must occur during neuronal differentiation because sprouting neurons exhibit yet another lectin binding pattern (K.H. Pfenninger, M.-F. Maylié-Pfenninger, L. B. Friedman, and P. Simkowitz, 1984, Dev. Biol. 106, 97-108). These results indicate that the commitment of ectodermal cells to diverging lineages (epidermis, neural crest, and tube) is reflected in their surface carbohydrates and occurs while they are still part of a continuous epithelial sheet. Furthermore, the plasmalemmal glycoconjugates of the ectoderm are developmentally regulated, and particularly dramatic changes in glycoconjugates expression are linked to neurulation.

Animals↗

PubMed1984

Ontogenetic expression of cell adhesion molecules: L-CAM is found in epithelia derived from the three primary germ layers.

Immunofluorescence techniques using specific antibodies against the liver cell adhesion molecule, L-CAM, were used to explore the appearance of L-CAM during early embryogenesis and organogenesis, as well as in adult tissue. Immunoblots of L-CAM from embryonic and adult organs indicated that molecules detected in each tissue were L-CAM, and that the antibodies were not simply detecting cross-reacting molecules. L-CAM was found in low levels on pregastrulation embryos. During gastrulation, the molecule remained present on ectoderm but was not detected on mesodermal and definitive endodermal cells. During neurulation, L-CAM disappeared from the neural ectoderm, in which staining for the neural cell adhesion molecule, N-CAM, had previously been shown to increase markedly. During organogenesis, L-CAM appeared in all endodermal structures, in ectoderm other than neural derivatives, in placodes, in extraembryonic ectoderm and endoderm, and in some mesodermal structures such as Wolffian ducts, oviduct, and kidney epithelium. Other mesodermal derivatives were not stained and the molecule was not detected in hemangioblastic areas of the lateral plate mesoderm nor in splanchnopleural derivatives such as spleen, adrenal glands, and gonads. During embryonic induction, for example, neurulation and in early kidney development, changes in L-CAM distribution were correlated with both locations and times of induction events. Analysis of distribution in the adult revealed that L-CAM was present in the stratum germinativum of the skin, in endodermally derived epithelia, in the female reproductive tract, and in the kidneys. In several fully differentiated glandular organs, L-CAM staining was restricted to basal or apical parts of the cell surface. When correlated with previous results obtained for N-CAM, these findings support the idea that local cell surface modulation of a small number of cell adhesion molecules may regulate other primary processes of development to yield specific patterns, both in early development and in organogenesis. Reflections of these patterns remain in adult life.

Animals↗

PubMed1986

An early developmental phase of pp60c-src expression in the neural ectoderm.

The expression of the normal cellular src protein (pp60c-src) was investigated in the early chick embryo during gastrulation and neurulation by immunoperoxidase staining using antisera, raised against bacterially expressed pp60v-src, that recognizes pp60c-src specifically in normal cells. During gastrulation pp60c-src immunoreactivity appeared primarily in the neural ectoderm and was much less prominent in the mesoderm, endoderm, and nonneural ectoderm. During neurulation pp60c-src immunoreactivity began to disappear from the wall of the closing neural tube so that by the completion of neural tube closure no specific pp60c-src immunoreactivity appeared in any of the neuroepithelial cells composing the neural tube. These studies reveal a developmental phase of pp60c-src expression even earlier than reported previously, when neuroepithelial cells of later embryos undergo terminal neuronal differentiation. These findings raise the possibility that pp60c-src may mediate two different differentiation signals in the neuronal lineage.

Age Factors↗

PubMed1987

Inductive interactions in the spatial and temporal restriction of lens-forming potential in embryonic ectoderm of Xenopus laevis.

The process of lens cell determination in amphibians is currently viewed as one involving a series of inductive interactions. On the basis of previous investigations, these interactions are thought to begin during gastrulation when the presumptive foregut endoderm and then the heart mesoderm come into contact with the presumptive lens ectoderm. This earlier period of induction is followed by the later interaction of the optic vesicle with the lens-forming ectoderm. Transplantation experiments were performed to determine the relative significance of the early and later periods of induction in the process of lens cell determination in the anuran Xenopus laevis. Various ectodermal tissues were transplanted either into the lens-forming region of open neural plate stage host embryos or over the newly formed optic vesicle of later neurula stage embryos. All transplanted tissues were labeled with the intracellular marker horseradish peroxidase to assess the exact origins of any induced lens structures. The results indicate that all nonneural ectodermal tissues have some lens-forming potential early during gastrulation; however, this potential is restricted to the lens-forming region, and perhaps nearby regions, later in development during the time of neurulation. Furthermore, the results show that the optic vesicle is not a substantial inductor of the lens in tissues that have not been previously exposed to the earlier series of inductive interactions that take place during gastrulation and neurulation. Since the optic vesicle does not appear to be a sufficient inductor of the lens, these earlier inductive interactions are, therefore, essential in the process of lens cell determination in Xenopus. These earlier inductive interactions lead to a steady increase in what may be called a lens-forming bias in the presumptive lens ectoderm during this period of development. The eventual loss in the ability of nonlens ventral ectoderm to respond to these lens inductors is presumably the result of other determinative processes that occur in this tissue.

Animals↗

PubMed1988

The first cleavage plane and the embryonic axis are determined by separate mechanisms in Xenopus laevis. I. Independence in undisturbed embryos.

We examined the spatial relationships between the meridian of sperm entry the plane of first cleavage, and the embryonic axis (defined by the neural groove) in eggs of Xenopus laevis. Direct measurement of the angular separations between these embryonic structures in gelatin-embedded eggs confirmed the classical conclusion that the sperm entry point and neural groove tend to form on opposite sides of the egg, and also revealed that the first cleavage plane has a nearly random orientation with respect to the neural groove. We next examined the distortion of the first cleavage plane that results from the normal processes of convergence and extension during gastrulation and neurulation. We permanently marked the first cleavage plane by injecting one blastomere of the two-cell embryo with a fluorescent lineage marker. At the start of gastrulation, the interface between the labeled and unlabeled regions was almost randomly oriented relative to the dorsal blastopore lip, confirming our first set of observations. In embryos with the interface less than 60 degrees to the plane passing through the midline of the dorsal lip, convergent movements of cells produced a confrontation of labeled and unlabeled cells along much of the dorsal midline. Thus, although the first cleavage plane and the bilateral plane were frequently not congruent, the morphogenetic movements of gastrulation and neurulation brought about an apparent congruence in many half-labeled embryos.

Animals↗

PubMed1989

Modulation of the embryotoxicity and cytotoxicity elicited by 7-hydroxy-2-acetylaminofluorene and acetaminophen via deacetylation.

Acetaminophen (APAP) and 7-hydroxy-2-acetylaminofluorene (7-OH-AAF) each produced a similar incidence of, as well as a qualitatively similar, abnormal closure of the anterior neuropore at similar concentrations when added to the medium of cultured rat embryos. At concentrations producing a 50-65% incidence of abnormal neurulation, the affected embryos displayed relatively complete embryonic development as assessed from measurements of protein, axial rotation, and embryonic length. The neural tube defect produced by these agents consisted of elevated neural folds remaining separated by approximately 45 degrees as well as the presence of a mitotically active neural epithelium. In contrast, the nonacetylated structures, p-aminophenol (PAP) and 7-hydroxyaminofluorene (7-OH-AF), were embryotoxic at concentrations 10-fold lower than the corresponding acetylated compounds; each produced a greater incidence of abnormal axial rotation and a greater decrease in embryonic protein than APAP or 7-OH-AAF. In addition, the embryos exposed to PAP or 7-OH-AF were morphologically and histologically dissimilar to those exposed to the acetylated compounds. The neural folds of the latter remained elevated and in apposition, but lacked complete fusion of the folds of neural epithelium and were accompanied by marked cytotoxicity. Addition of active deacetylase sources (guinea pig liver microsomes or commercially obtained, purified carboxylic-ester hydrolase) to the culture medium of conceptuses exposed to 7-OH-AAF or APAP resulted in an increased embryotoxicity which was indistinguishable from that produced by the nonacetylated compounds alone. The increases in toxicity were effectively blocked by the addition of paraoxon, indicating that catalysis of the deacetylation of APAP and 7-OH-AAF was the causative factor. No evidence could be found for deacetylation of 7-OH-AAF or APAP mediated by the Day 10 conceptus itself. When examined for cytotoxicity in F9 embryonal carcinoma cells, APAP and 7-OH-AAF each produced observable cell death only if reduced glutathione (GSH) had previously been depleted and if a deacetylase source were present; this cytotoxicity was also blocked by addition of paraoxon. The nonacetylated metabolites were directly cytotoxic, although GSH depletion greatly increased the incidence of cell death. Therefore, deacetylation of APAP and 7-OH-AAF produced an increase in generalized embryotoxicity and cytotoxicity relative to abnormal neurulation, suggesting that APAP and 7-OH-AAF are capable of eliciting neural tube defects via a mechanism(s) that is distinguishable from the generalized embryotoxicity or cytotoxicity produced by their nonacetylated counterparts.

2-Acetylaminofluorene↗

PubMed1995

Down-regulation of ornithine decarboxylase by an increased degradation of the enzyme during gastrulation of Xenopus laevis.

The present study was designed to analyze the regulation of the levels of the polyamines and their biosynthetic enzymes during embryonic development of Xenopus laevis. The activity of ornithine decarboxylase (ODC), a rate-controlling enzyme in polyamine biosynthesis, is elevated until, during gastrulation, there is a precipitous drop in activity. This is not attributable to a decrease in ODC mRNA content and polysome profiles reveal no apparent decrease in ODC message associated with polysomes. ODC synthesis seems to be maintained at a low, relatively constant rate until neurulation whereupon ribosome loading of ODC mRNA increases. During gastrulation the rate of ODC degradation increases dramatically, which can account for the decrease in ODC. S-Adenosylmethionine decarboxylase (AdoMetDC), another rate-controlling enzyme in polyamine biosynthesis, shows a low and constant activity from cleavage to neurulation. Subsequently, the AdoMetDC activity increases dramatically. The changes in AdoMetDC activity parallel the changes in AdoMetDC mRNA levels, suggesting a transcriptional control of AdoMetDC expression during this development period. The activities of ODC and AdoMetDC produce a steady increase in putrescine and spermidine content of the embryo. The spermine content also increases until gastrulation, but then decreases until the tailbud stage.

Adenosylmethionine Decarboxylase↗

PubMed1993

Isolation of cDNAs for two closely related members of the axolotl Wnt family, Awnt-5A and Awnt-5B, and analysis of their expression during development.

To characterize molecular interactions between cells in the early amphibian embryo, we have isolated cDNAs for two members of the axolotl (Ambystoma mexicanum) Wnt family, Awnt-5A and Awnt-5B. The encoded proteins share 83% amino acid identity. Using a reverse transcription-polymerase chain reaction (RT-PCR) assay, we find that Awnt-5A transcripts are abundant in the blastula until gastrulation, barely detectable during gastrulation, and increase again during neurulation. They are detected throughout the remaining development and in hatched larvae. In contrast, transcripts for Awnt-5B are undetectable in the blastula. They appear with gastrulation, are present throughout neurulation and organogenesis, and decrease to barely detectable levels in hatched larvae. PCR reactions performed using cDNA library-phage DNA templates derived from whole neurulae versus embryos with the neuroectoderm removed suggest that, in the neurula, Awnt-5A transcripts are present in neuroectodermal as well as non-neuroectodermal tissues while Awnt-5B mRNAs are predominantly localized in the neuroectoderm. To localize Awnt-5A expression in embryos before gastrulation, early gastrulae were dissected by cutting along the animal-vegetal and future dorso-ventral axes and analyzed by RT-PCR. At this early stage, Awnt-5A transcripts appear to be predominantly localized in the dorso-vegetal region of the embryo. These results suggest that the two closely related Awnt-5 genes participate in different morphogenetic processes during early axolotl development.

Ambystoma mexicanum↗

PubMed2006

Embryonic expression of zebrafish AMPA receptor genes: zygotic gria2alpha expression initiates at the midblastula transition.

The AMPA-preferring receptors (AMPARs) mediate rapid excitatory synaptic transmission in the central nervous system of vertebrates. Expression profiles of 8 AMPAR genes were studied by RT-PCR analyses to elucidate the properties of AMPARs during early zebrafish development. Transcripts of all AMPAR genes are detected at the time of fertilization, suggesting maternal transcriptions of zebrafish AMPAR genes. The amounts of gria1 and gria2 transcripts are several-fold higher than that of gria3 and gria4 between 10 and 72 hpf (hour postfertilization). The edited gria2alpha transcript decreases during gastrulation period, suggesting that zygotic expression of gria2alpha begins around the time of midblastula transition. Relative to the amount of beta-actin, the amounts of AMPAR transcripts increase significantly after the completion of neurulation. The amounts of gria2 transcripts exceed the total amounts of the remaining AMPAR transcripts after 36 hpf, suggesting increases in the representation of low Ca2+ permeable AMPARs during neuronal maturation. Many but not all of the known mammalian protein-protein interaction motifs are preserved in the C-terminal domains (CTD) of zebrafish AMPARs. Before 16 hpf, the embryos express predominantly the alternative splice forms encoding longer CTD. Representations of the short CTD splice forms of gria2 and gria4alpha increase after 24 hpf, when neurulation is nearly completed.

Animals↗

PubMed2004

Imaging in spine and spinal cord malformations.

Spinal and spinal cord malformations are collectively named spinal dysraphisms. They arise from defects occurring in the early embryological stages of gastrulation (weeks 2-3), primary neurulation (weeks 3-4), and secondary neurulation (weeks 5-6). Spinal dysraphisms are categorized into open spinal dysraphisms (OSDs), in which there is exposure of abnormal nervous tissues through a skin defect, and closed spinal dysraphisms (CSD), in which there is a continuous skin coverage to the underlying malformation. Open spinal dysraphisms basically include myelomeningocele and other rare abnormalities such as myelocele and hemimyelo(meningo)cele. Closed spinal dysraphisms are further categorized based on the association with low-back subcutaneous masses. Closed spinal dysraphisms with mass are represented by lipomyelocele, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise simple dysraphic states (tight filum terminale, filar and intradural lipomas, persistent terminal ventricle, and dermal sinuses) and complex dysraphic states. The latter category further comprises defects of midline notochordal integration (basically represented by diastematomyelia) and defects of segmental notochordal formation (represented by caudal agenesis and spinal segmental dysgenesis). Magnetic resonance imaging (MRI) is the preferred modality for imaging these complex abnormalities. The use of the aforementioned classification scheme is greatly helpful to make the diagnosis.

Humans↗

PubMed2005

Expression of Panza, an alpha2-macroglobulin, in a restricted dorsal domain of the primitive gut in Xenopus laevis.

Alpha2-macroglobulin is a major serum protein with diverse functions, including inhibition of protease activity and binding of growth factors, cytokines, and disease factors. We have cloned and characterized Panza, a new Xenopus laevis alpha2-macroglobulin. Panza has 56-60% amino acid similarity with previously identified Xenopus, mouse, rat and human alpha2-macroglobulins, indicating that Panza is a new member of the alpha2-macroglobulin family. Panza mRNA is first detected at the beginning of neurulation in the dorsal endoderm lining the primitive gut (archenteron roof). At the completion of neurulation and continuing through the late tadpole stage, Panza is restricted to a dorsal domain of the gut endoderm adjacent to the notochord and extending along the entire anterior-posterior axis. With outgrowth of the tailbud, Panza expression persists in the chordaneural hinge at the posterior end of the differentiating notochord and extends into the floor plate of the posterior neural tube. As gut coiling commences, Panza expression is initiated in the liver, and the dorsal domain of Panza expression becomes limited to the midgut and hindgut. With further gut coiling, strong Panza expression persists in the liver, but is lost from other regions of the gut. The expression of Panza in endodermal cells adjacent to the notochord points to a potential role for Panza in signal modulation and/or morphogenesis of the primitive gut.

Amino Acid Sequence↗