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G Steding

Publications and source records attributed to G Steding.

At least 19 recordsLinked to original sources

The scoliosis (sco) mouse: a new allele of Pax1.

We describe the spontaneous mutant mouse scoliosis (sco) that carries a new allele of Pax1 (un-i, undulated intermediate). The Pax1(un-i) allele is lacking the 5'-flanking region and exon 1 to 4 which is mapped to nt -2636 to -640 and -272 to 4271 of the Pax1 gene. Homozygous mice show a mild form of the known phenotypes of other Pax1 mutants. Adult mice have a lumbar scoliosis and kinky tails. In homozygous embryos the skeleton ossifies early, ossification centers of the vertebral bodies are fused with the ossification centers of the pedicles. Neural arches and spinous processes are underdeveloped but the pedicles and transverse processes are overdeveloped which is in contrast to other Pax1 mutants. In the scapula, the acromion is missing and the deltoid tuberosity of the proximal humerus is shortened and thickened. Among the inner organs the thymus development is affected. In late embryos, the thymus is small and thymocyte numbers are reduced. T-cell development from CD4- and CD8- double negative (DN) to CD4+ and CD8+ double positive (DP) is decelerated. The percentage of CD90+ cells is also reduced but in contrast to other Pax1 mutants no alteration of the expression level of the CD90 (Thy-1) could be found.

Animals↗

[Is there new knowledge on embryology and functional anatomy of human mimetic muscles and the upper lip? A contribution to point selection, skin incision and muscle reconstruction in primary lip-nose reconstruction of uni- and bilateral lip-jaw-palatal clefts].

BACKGROUND: The great variety of primary cheiloplastic procedures in CLP patients shows that there is disagreement regarding the embryological development of this part of the face, the point selection, skin incision philosophy, and the macroscopic and microscopic functional anatomy of the human muscles of facial expression. We suppose from findings in Asian and African populations that the real embryological development of the upper lip differs from current textbook descriptions. Our own anatomical and embryological investigations serve as a basis for a critical discussion of different techniques of muscle reconstruction, point selection, and skin incision and for a description of an embryologically, functionally, and anatomically oriented operation technique for different entities of CLP. METHODS: The findings of this study result from investigations of the embryonal and early fetal development from the 26th to the 112th i.u. day in REM pictures of the Anatomical Institute of the University of Göttingen (n = 8) and serial histological investigations of the Carnegie and Hooker-Humphrey Collections at the Armed Forces Institute of Pathology, Washington, D.C. (n = 40). Furthermore, we carried out microsurgical dissections of the muscles of facial expression, the osseous and cartilaginous parts of the nose, and the midfacial sutures in two adult heads without congenital disorders and one newborn head with a primary unilateral complete cleft of the lip and alveolus. RESULTS AND DISCUSSION: The formation of the lower third of the upper lip is the result of contact of the maxillary bulges in the midline below the prolabium. According to this finding, the point selections and skin incisions have to be modified in the midline region in different types of uni- and bilateral CLP. Our technique of primary dissection, reorientation, and suturing of the muscles of facial expression is presented. The muscle reconstruction has to be performed independently from the skin preparation.

Cleft Lip↗

Horseshoe lung: report on a new variant--"inverted" horseshoe lung--with embryological reflections on the formal pathogenesis of horseshoe lungs.

The term "horseshoe lung" is used to describe a rare congenital anomaly of the lungs that is characterized by the presence of a midline isthmus of pulmonary parenchyma connecting the posterobasal regions of the right and left lungs. Since the introduction of the term horseshoe lung in the 1960's, almost 40 cases have been reported in the literature. In all these cases, the right and left lungs were joined in their posterobasal regions, the situation resembling that found in horseshoe kidneys. Here we present a case of connection between the right and left lungs found during necropsy of a human fetus. In this case, a midline isthmus of pulmonary parenchyma covered by visceral pleura joined the apical regions of the right and left lungs behind the trachea and esophagus. This connection resulted in a "horseshoe"-shaped lung in which the "horseshoe" was turned by 180 degrees compared to classical cases of horseshoe lung. To the best of our knowledge, this is the first report on an "inverted" horseshoe lung. Embryological reflections on the formal pathogenesis of inverted and classical horseshoe lungs are presented.

Abortion, Spontaneous↗

[Anomalies of muscles in the parotid area--a contribution to parotid surgery].

A Contribution to Parotid Surgery. Anomalous muscles in the parotid area may be derived from platysma fibres migrating to ventral or dorsal. We describe a M. occipito-parotideus with its origin in the back of the neck crossing the upper part of the M. sternocleidomastoideus and inserting the fascia parotidea. This muscle may irritate the surgeon preparing the muscular landmarks of parotid and facial nerve surgery.

Facial Muscles↗

Targeted disruption of the Insl3 gene causes bilateral cryptorchidism.

The sexual dimorphic position of the gonads in mammals is dependent on differential development of two ligaments, the cranial suspensory ligament (CSL) and the gubernaculum. During male embryogenesis, outgrowth of the gubernaculum and regression of the CSL result in transabdominal descent of the testes, whereas in the female, development of the CSL in conjunction with failure of the gubernaculum development holds the ovaries in a position lateral to the kidneys. Several lines of evidence suggest that regression of the CSL and induction of gubernaculum development are mediated by testosterone and a yet unidentified testicular factor, respectively. The Insl3 gene (originally designated Ley I-L), a member of the insulin-like superfamily, is specifically expressed in Leydig cells of the fetal and postnatal testis and in theca cells of the postnatal ovary. Here we show that male mice homozygous for a targeted deletion of the Insl3 locus exhibit bilateral cryptorchidism with free moving testes and genital ducts. These malformations are due to failure of gubernaculum development during embryogenesis. In double-mutant male mice for Insl3 and androgen receptor genes, testes are positioned adjacent to the kidneys and steadied in the abdomen by the CSL. These findings demonstrate, that the Insl3 induces gubernaculum development in an androgen-independent way, while androgen-mediated regression of the CSL occurs independently from Insl3.

Androgens↗

Complete transposition in a chick embryo demonstrated by scanning electron microscopy.

Chick embryos are frequently used as animal models when researching the developing heart. In the past, every attempt to induce complete transposition (the combination of concordant atrioventricular and discordant ventriculo-arterial connections) failed in chicks, suggesting that it might be impossible to develop a chicken model for this malformation. We demonstrate, to the best of our knowledge, the first well-documented case of complete transposition occurring in the chick.

Animals↗

The formal pathogenesis of isolated common carotid or innominate arteries: the concept of malseptation of the aortic sac.

Deficient connections (= isolation) of the innominate artery or the common carotid artery to the aorta are rare congenital anomalies of the human aortic arch complex that are usually associated with a patent vascular connection between the isolated artery and a pulmonary artery. In the present study we demonstrate chick fetuses with a corresponding anomaly, the isolation of the brachiocephalic artery. In our chick fetuses the left brachiocephalic artery did not arise from the aortic arch, but was connected to the pulmonary trunk proximal (upstream) to the patent left and right ductus arteriosus. These cases are of interest because the presence of a congenital pulmonary-systemic arterial connection proximal (upstream) to the ductus arteriosus cannot be explained by the traditional concept of the morphogenesis of the aortic arch complex. The development of the normal and abnormal branching patterns of the aortic arch arteries is traditionally explained by transformation of the primitive embryonic pharyngeal arch arterial system due to obliteration of some of its vascular segments. Based on this concept, the isolation of an aortic arch artery can be explained by obliteration of vascular segments proximal and distal to this artery, whereas its connection to a pulmonary artery can be explained only by deficient obliteration (persistence) of the distal portion of the right or left sixth pharyngeal arch artery. The connecting "vascular segment" between an isolated aortic arch artery and the pulmonary circulation, therefore, is traditionally interpreted as a patent ductus arteriosus. The formal pathogenesis of congenital pulmonary-systemic arterial connections proximal (upstream) to the ductus arteriosus is discussed. The presented cases of isolation of the brachiocephalic artery are explained by disturbances in the partition of the embryonic aortic sac, possibly due to abnormal development of the "cardiac" neural crest.

Animals↗

Embryological observations on the formal pathogenesis of double-outlet left ventricle with a right-ventricular infundibulum.

The term 'double-outlet left ventricle' (DOLV) denotes congenital heart malformations in which the aorta and the pulmonary trunk both arise entirely or predominantly above the morphologically left ventricle. In the past, the formal pathogenesis of DOLV was explained by an abnormal topogenesis of the origin of the great arteries, which could be caused by an excessive leftward shift of the embryonic conotruncus, or by errors in differential conal growth or absorption. However, modern embryological and pathological research is casting doubt on the validity of these concepts. In the present paper we demonstrate a case of DOLV found in a chick fetus. In this heart the main derivatives of the embryonic conotruncus (right-ventricular infundibulum and proximal portions of the great arteries) principally are in the normal position and of normal dimensions. The anomaly leading to DOLV under these conditions is a misalignment of the ventricular septum. The subarterial portion of the ventricular septum above the crista supraventricularis is not oriented in the normal oblique plane between the pulmonary and aortic valve, but is oriented in a frontal plane anterior to the origin of both great arteries. The consequences of this anomaly are the separation of the right-ventricular infundibulum from the origin of both great vessels (DOLV) and a lack of continuity between the malpositioned portion of the ventricular septum (posterior wall of the right-ventricular infundibulum) and a septum dividing the semilunar valve level. The infundibulum of the right ventricle is derived from the upstream portion of the embryonic conotruncus (conus) whereas the semilunar valves and great arteries are derived from its downstream protion (truncus arteriosus) and the aortic sac. Therefore, our findings suggest that the division of the conotruncus is performed by at least two different septa, one dividing the conus and another dividing the truncus arteriosus and aortic sac. The misalignment of the ventricular septum leading to the presented type of DOLV could result from a misalignment of the septal anlagen of the embryonic conus, the conus ridges. Our findings are discussed with respect to human cases of DOLV and with respect to a recent concept on the septation of the embryonic conotruncus.

Animals↗

Experimental study on the significance of abnormal cardiac looping for the development of cardiovascular anomalies in neural crest-ablated chick embryos.

During normal development, ectomesenchyme from the cardiac neural crest migrates to pharyngeal arches 3, 4, 6 and the developing heart. It participates in the formation of the aorticopulmonary septum and the wall of the great arteries. Removal of the cardiac neural crest resulted in anomalies of the great arteries and in two categories of severe heart defects: (1) outflow septation defects of the persistent truncus arteriosus (PTA) type, (2) alignment defects. It has been hypothesized that PTA occurs if the number of cardiac neural crest cells is reduced below a level critical for complete formation of the aorticopulmonary septum. Alignment defects would be indirect consequences of neural crest defects, possibly caused by altered blood flow in the pharyngeal arch region. We found that these concepts were not in agreement with some experimental facts reported previously, so we considered whether there could be other mechanisms responsible for the heart defects described. To investigate whether mechanical interference with cardiac looping could possibly contribute to the pathogenesis of these anomalies, we removed the entire cardiac neural crest in chick embryos with micro-needles. Postoperative development was checked during cardiac looping and after normal completion of cardiac septation. Our data suggested that abnormal cardiac looping did not contribute to the pathogenesis of the aortic arch artery anomalies and PTA. With respect to the alignment heart defects, we could not elucidate the role of looping anomalies because we did not observe such heart defects. Moreover, PTA occurred only in 28% of survivors. This finding conflicts with previous studies where extensive ablation of the cardiac neural crest has led to a high incidence of PTA (73-100% of survivors). The possible reasons for this discrepancy are discussed. It is shown that the use of different microsurgical techniques (mechanical cutting/microcautery) may be responsible for the different incidence of PTA. We speculate that microcautery hampers a normal complete repair of neural crest defects, possibly by release of abnormally high levels of growth factors.

Animals↗

The role of extracardiac factors in normal and abnormal development of the chick embryo heart: cranial flexure and ventral thoracic wall.

The present study was designed to investigate whether the formation of the cranial flexure is involved in the normal positional changes of the embryonic heart tube that occur during its transformation from the c- to s-shaped loop. For this purpose, the formation of the cranial flexure was locally suppressed in chick embryos by introducing a straight hair into the neural canal. In the experimental embryos, prevention of cranial flexure did not suppress the normal positional changes of the heart tube. However, other anomalies in the looping of the heart tube were frequently observed. These anomalies were caused by alterations in the formation of the ventral thoracic wall, which in turn seemed to be related not to the prevention of the cranial flexure but rather to accidental injuries during the implantation of the hair. In the embryos with abnormal looping of the heart tube, the incidence of delayed/defective septation of the heart was significantly higher than in embryos with normal looping. These results show that in the chick embryo: (1) cranial flexure is not involved in normal positional changes of the heart loop; (2) manipulations at the head region of the embryo can unintentionally result in developmental disorders of the ventral thoracic wall; (3) such disorders can result in congenital heart defects through mechanical interference with normal looping of the embryonic heart. The possible significance of these findings for the evaluation of experimental studies of chick embryos is discussed in the context of anomalies observed after surgical ablation of the premigratory cranial neural crest.

Animals↗

Embryological observations on the morphogenesis of double-outlet right ventricle with subaortic ventricular septal defect and normal arrangement of the great arteries.

Double-outlet ventricle (DORV) is generally regarded as a congenital heart defect resulting from impaired morphogenesis of either the outflow portion (conotruncus) or the conoventricular flange (crista prima) of the embryonic heart. However, we demonstrate in this study chicken fetal hearts with DORV in which the conotruncal derivatives (great arteries and subpulmonary part of the ventricular septum = conus septum) and the region of the crista prima are normally developed. The anomalies leading to DORV under these conditions are found at the atrioventricular region. The posterior-anterior axis of the tricuspid orifice is not directed to the right anterior but to the left anterior side of the heart. Thereby the posterior connection line between the muscular ventricular septum and conus septum, which usually follows the left margin of the tricuspid orifice, is not connected to the right portion of the conus septum but instead is directed towards the left portion of the conus septum. In consequence of the abnormal connection between the muscular ventricular septum and the conus septum, the interventricular foramen is formed at the left side of the subaortic flow path. The subaortic flow path arises from the right ventricle. These findings show that DORV can result not only from impaired development of the conotruncus or conoventricular flange, but also from abnormal development of the atrioventricular region. We suggest distinguishing between conotruncal, conoventricular (crista prima), and atrioventricular types of DORV.

Animals↗

Formation of the cervical flexure: an experimental study on chick embryos.

It has been proposed that the cervical flexure of vertebrate embryos arises from the normal morphogenesis of the heart. This hypothesis is based on experiments in which the heart tube is removed or disrupted in early chick embryos. It has been reported that, in normal atmosphere, these embryos continued normal morphogenesis except for cervical flexure formation. In the present study, we performed similar experiments. In contrast to previous work, however, only one set of our heart-deprived chick embryos was reincubated in normal air. The other sets were reincubated in oxygen-enriched air. Under normoxia, heart removal resulted not only in prevention of the cervical flexure, but also in mesenchymal defects, and in a remarkable hypoplasia of the craniocervical region. Under hyperoxia, heart-deprived embryos developed no severe mesenchymal defects and the growth of the upper body portion was more normal, with the hypoplasia confined to the cranial region. The formation of the cervical flexure was now normalized. These results show that cervical flexure formation is not directly dependent on normal morphogenesis of the heart, but does depend on a sufficient oxygen supply to the cervical region. During early development, the cranio-cervical region of a chick embryo is more sensitive to circulatory failure than the trunk.

Animals↗

Kinetics and differentiation of somite cells forming the vertebral column: studies on human and chick embryos.

We have studied the kinetics of somite cells with an antibody against proliferating cell nuclear antigen (PCNA/cyclin) in human and chick embryos, and with the BrdU anti-BrdU method in chick embryos, to investigate whether the metameric pattern of the developing vertebral column can be explained by different proliferation rates. Furthermore we applied antibodies against differentiation markers of chondrogenic and myogenic cells of the somites in order to study the correlation between proliferation and differentiation. There are no principal differences in the proliferation pattern of the vertebral column between human and chick embryos. In all stages examined, the cell density is higher in the caudal sclerotome halves than in the cranial halves. Laterally, the caudal sclerotome halves, which give rise to the neural arches, are characterized by a higher proliferative activity than the cranial halves. Although there is a high variability, the labelling indices show significant differences between the two halves with both proliferation markers. With the onset of chondrogenic differentiation, only the perichondrial cells retain a high proliferation rate. During fetal development, the neural arches and their processes grow appositionally. Even at the earliest stages, there is practically no immunostaining for PCNA or BrdU in the desmin-positive myotome cells of human and chick embryos. Axially, a higher proliferation rate is found in the condensed mesenchyme of the anlagen of the intervertebral discs than in the anlagen of the vertebral bodies. During fetal development, cells at the borders between vertebral bodies and intervertebral discs proliferate, indicating appositional growth.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Correlation between the embryonic head flexures and cardiac development. An experimental study in chick embryos.

The aim of the present study is to examine whether the formation of the cranial and cervical flexures is involved in the process of cardiac looping, and whether looping anomalies are causally involved in the development of cardiac malformations. For this purpose, the formation of the cranial and cervical flexures was experimentally suppressed in chick embryos by introducing a straight human hair into the neural tube. In the experimental embryos, the absence of the cervical flexure, alone or in combination with a reduced cranial flexure, was always associated with anomalies in the looping of the tubular heart. The convergence of the primary distant venous and arterial ends of the heart, as well as the normal movement of the ventricular region from its original position, cranial and ventral from the cardiac inflow, to its final position caudal to the presumptive atria, was suppressed to an extent related to the degree to which the formation of the flexures was prevented. Positional immaturity of the heart loop (increased distance between its inflow and outflow, and cranio-ventral position of the ventricular region) was associated with incomplete deformations (reduced angulations) of the cardiac wall at the atrioventricular or conoventricular junctional areas. Reduced angulations were associated with the hypoplasia of the anlagen of the cardiac septa at the level of the angulation (av-cushions, conal ridges). Hypoplasia of these anlagen was followed by incomplete or absent fusion of their opposite free edges, which finally resulted in atrioventricular or ventricular septal defects. These results show that the convergence of the venous and arterial ends of the tubular heart and the caudo-dorsal movement of its ventricular region are related to the formation of the cervical flexure, and that the mesenchymal septa of the heart seem to develop in response to deformations of the embryonic heart, which are generated by the process of cardiac looping. Therefore, the positional and morphological changes of the looping heart are regarded as playing a key role in the process of normal and abnormal morphogenesis of the heart.

Abnormalities, Multiple↗

Developmental aspects of the sinus valves and the sinus venosus septum of the right atrium in human embryos.

In 32 human embryos from 5 to 27 mm of length, stages 13 to 23 (according to the Carnegie system of stages), the contributions of the sinus venosus septum and the right sinus valve of the right atrium to the formation of the Eustachian and Thebesian valve were examined by scanning electron microscopy. The sinus septum takes part in the subdivision of the right sinus valve into the Eustachian and the Thebesian valves. From its first origin the sinus septum forms a septal structure between the orifices of the right hepatic vein (hepatic portion of the inferior caval vein), the precursor of the inferior caval vein, and the left horn of the sinus venosus, the precursor of the coronary sinus. Before the incorporation of the sinus venosus into the right atrium, it has an intra-sinusal position, and extends between the bases of the left and the right sinus valve. During the incorporation of the sinus venosus into the right atrium the sinus septum receives an intra-atrial position, and its positional relationships to the sinus valves and the orifices of the corresponding veins remain unchanged in principle. Due to the connection between the sinus septum and the right sinus valve, after completion of the incorporation of the sinus, the superior portion of the right sinus valve branches y-like into a lateral limb, (i.e. its original inferior portion) and into a medial limb, (i.e. the sinus septum).(ABSTRACT TRUNCATED AT 250 WORDS)

Heart Septum↗

[The orientation of prisms in the dental enamel of human permanent teeth].

Enamel of human permanent teeth was sectioned and ground with 2 planes perpendicular to each other extending centrifugally from the dentino-enamel-junction to the crown surface. Prisms were made visible by acid etching before evaluation under the SEM. In the vicinity of the dentino-enamel-junction more prisms were found to be cut longitudinally, while close to the crown surface more prisms were cut transversely. In the perpendicularly ground plane the corresponding prisms were seen to deviate the more from the centrifugal orientation the more close they came to the crown surface. In a geometric model the angle under which the prisms deviate from the centrifugal orientation was calculated in dependence from the distance to the dentino-enamel-junction. The results correspond with the SEM-findings. We conclude that--since the prism diameter is today known to be constant--the form of the enamel mantle is created by a specific orientation of the prisms: They stand perpendicular at the dentino-enamel-junction, and the farther away they run towards the periphery the more they deviate from the perpendicular path. By this increasingly oblique orientation of the prisms the volume increment of the enamel mantle is created. The maximal angle of deviation is found morphologically and mathematically between 60 degrees-70 degrees at the crown surface. This arrangement of the prisms demonstrated by us is now seen to be the reason for erroneous assumptions about an increase of the prism diameter when ground sections were used. Because the prisms are oriented more and more oblique towards the periphery, correspondingly larger effective diameters must be produced while making ground sections tangential to the crown surface.

Dental Enamel↗

[Early development of human tooth germs including the surrounding structures].

It could be shown by means of 3-dimensional reconstructions made from serial sections of human embryos, that the dental lamina of the lower jaw as well as that of the upper jaw is spatially impeded at its distal ends by the presence of the mandible and Meckel's cartilage. This spatial impediment of the odontogenetic epithelium can be seen to be of influence during the formation of the tooth buds.

Humans↗

[The orientation of the enamel prisms at the enamel surface].

The angulation that prisms obtain with the surface in human permanent teeth was analysed on broken enamel by means of the scanning electron microscope. In the cervical region the prisms end nearly perpendicular towards the surface, while the angle becomes more and more acute towards the occlusal region of the crown. In the cuspal area the deviation from the perpendicular direction of the prisms approaches 70 degrees. The size discrepancy between the inner and the outer surface of the enamel mantle can be explained by the more or less angulated position of the prisms.

Bicuspid↗