bluuebunny/crossref_metadata_2025_split huggingface dataset:
Centronucleated Myofibers Having Also Peripheral Nuclei in Rectus Femoris Muscles of Muscular Dystrophic Mice
Authors: TOTSUKA Tsuyoshi 
Published: May 2008

Abstract The diameter distribution was compared between centronucleated and non‐centronucleated myofibers on a cross‐sectional specimen of the hindleg rectus femoris muscle from muscular dystrophic (dy/dy) mice aged from 17 to 120 days. At any age examined, it was very similar in centronucleated and non‐centronucleated myofibers, and the proportion in number of the former to the latter was so high as about 1/3 to 1 in spite that it was determined on a single cross section of 5 jum thick for each muscle. The histochemical study showed that central nuclei were observable apparently in any type of myofibers stained densely, palely or intermediately with NADH dehydrogenase or PAS. In addition, it was found that a considerable number of centronucleated myofibers had also peripheral nuclei and that the central nuclei tended to migrate neither toward the center nor toward the periphery in the myofiber with age or growth. These findings are inconsistent with the widely‐accepted view that the centronucleated myofibers in dystrophic muscles are the regenerated ones which are abortive.The pathological implication of the central nucleation was discussed with reference to the bone‐muscle imbalance hypothesis previously postulated by the present author as the pathogenesis of mouse muscular dystrophy.
Effects of H‐2 Histocompatibility Gene Complex on Urethan and Ethylnitrosourea (ENU)‐lnduced Malformations in Mice
Authors: NITO Shinji , OKANIWA Azusa , MORIWAKI Kazuo 
Published: May 2008

Abstract The genetic effects of the H‐2 histocompatibility gene complex on the frequencies of urethan and ethylnitrosourea (ENU)‐induced malformations in prenatal fetuses of mice were examined, and a new functional gene linked to the H‐2 complex on chromosome 17 was mapped.The H‐2 congenic pair strains of mouse, BIO.A and C57BL/10 (BIO), were tested for susceptibility to urethan‐induced external malformations in fetuses. The BIO.A strain expressing the H‐2a haplotype characteristically showed a high susceptibility to urethan‐induced Polydactyly and kinky tail. However, the BIO strain expressing the H‐2b haplotype was resistant to urethan‐induced malformation. The same tendency was observed for ENU‐induced malformations. Thus, susceptibilities to urethan and ENU‐induced external malformation must be affected by a gene linked to the H‐2 complex.In a mapping study using BIO.A recombinant mouse strains, BIO.A (5R) was sensitive, while BIO.A (2R) and B10.A (4R) were resistant to urethan‐induced external malformations. Variations in the susceptibility to teratogenicity of urethan in BIO.A recombinant strains indicated that a new functional gene located between the H‐2 S region and the Upg‐1 locus on chromosome 17 was involved in regulation of susceptibility.
An Autopsy Case of Tracheal Agenesis with Broncho‐esophageal Fistula
Authors: SUGIYAMA Eiichiro , IKEDA Takayoshi , MAEDA Hiroshi , MATSUO Takeshi , MATSUMOTO Masaru 
Published: May 2008

Abstract True incidence of this malformation is probably greater than that reported since the definitive diagnosis has been made at autopsy in most cases. Various hypotheses on the pathogenesis of tracheal agenesis have been proposed but they are still controversial.In this report, we present a case of tracheal agenesis with a broncho‐esophageal fistula and discuss the formal genesis.
Evaluation of the Effects of Paternal Drug/Chemical Exposure on the Offspring
Authors: ADAMS Perrie M. 
Published: May 2008

Abstract A number of different approaches to the study of the effects of male‐mediated drug or chemical insult on the subsequent offspring have been used. Typically a modified dominant lethal protocol has been used with the offspring allowed to go to term and the functional development assessed at specific postnatal ages. The present review examines the importance of the mating times for identification of the stages of spermatogenesis most sensitive to insult and the possible mechanisms involved in male‐mediated transmission of the effect of toxic exposure to the offspring.The functional assessments demonstrating a paternal‐mediation of drug and/or chemical exposure have included motor coordination, reflexive behavior, locomotor activity and associative learning. The importance of the mating sequence, assessment methods and interpretation alternatives must be considered in designing studies to identify the extent of the risk to the male genome of drug/chemical exposure as reflected in functional deficits to the subsequent offspring.
Chromosome Analysis in Preimplantation Stage Embryos of Non‐Obese Diabetic (NOD) Mice
Authors: TATEWAKI Reiko , OTANI Hiroki , TANAKA Osamu , KITADA Jin‐ichi 
Published: May 2008

Abstract Congenital malformations such as exencephaly occur at a high incidence in non‐obese diabetic (NOD) mouse embryos (Otani et al., 1987a). Using in vitro culture and embryo transfer, genetic predispositions as well as environmental factors were suggested to be the causes (Tatewaki et al., 1987).Chromosomes of preimplantation embryos, cultured to transfer mutually between non‐obese diabetic (NOD) and ICR mice, were analyzed as a clue to elucidate the cause of developmental anomalies. Among these preimplantation embryos, aneuploidy, mixoploidy and structural anomalies were found, but in incidence of chromosomal anomalies there was no significant difference, statistically, between preimplantation embryos from diabetic mothers and those from non‐diabetic mothers. These findings are considered to show that no increase in developmental abnormality in chromosomes occurs during the preimplantation stage.
Fetal Heart Malformations in Experimental Hyperphenylalaninemia in Pregnant Rats
Authors: SUYAMA Itsujin , TANI Mayumi , MATSUMURA Masahiro , ISSHIKI Gen , OKANO Yoshiyuki , OURA Toshiaki , NISHI‐MURA Kouichi 
Published: May 2008

Abstract Sprague‐Dawley rats were given L‐phenylalanine intraperitoneally from the 8th to 11th day of pregnancy. The hearts of the fetuses were examined on the 21st day of pregnancy. We found that the group given the higher doses of L‐phenylalanine had significantly more heart defects than the group given the lower dose and the controls. Ventricular septal defect was found in 80 % of the fetuses with congenital deformities of the heart. [14C] Leucine uptake by the embryos was significantly lower in the group loaded with L‐phenylalanine than in the controls.The activity of thymidine kinase, one of the rate‐limiting enzymes in DNA synthesis, was significantly lower in the brain and heart tissues of the young rats loaded with L‐phenylalanine than in the controls.Thus, when blood levels of phenylalanine are high in early pregnancy, an amino acid imbalance in the embryo occurs during fetal organogenesis. Also, thymidine kinase decreases, which may hinder DNA synthesis. Both of these mechanisms seem to be involved in the higher incidence of fetal heart malformations in maternal phenylketonuria.
Effect of Prenatal Methylazoxymethanol Acetate Exposure on the Motor Behavior of the Rat Offspring
Authors: YAMAMOTO Yoshitame , TANIMURA Takashi 
Published: May 2008

Abstract Crj:CD (Sprague‐Dawley) rats were treated intraperitoneally with methylazoxymethanol acetate (MAM) at 0 and 30 mg/kg on day 13 of gestation and were allowed to deliver. On day 4 postpartum, the litter size was adjusted to 8 with an equal sex distribution. Two males and females from each litter were tested between 3 and 5 weeks of age for open field activity and rotorod performance. At week 7 postpartum, all offspring were sacrificed and examined for brain anomalies.The open field activity of MAM treated offspring tended to increase; number of ambulations and rearings were significantly increased at 3 weeks of age in male offspring treated with MAM when compared to those of male controls. At 3 weeks of age, the male offspring in the MAM treated group showed significantly reduced rotorod performance when compared to that in the control. Severe reductions of the cerebral hemispheres were observed at 7 weeks of age in the male and female offspring treated with MAM.These results indicate that the motor behavior of offspring is significantly impaired at weaning when they were exposed to MAM prenatally. The effects were more severe for males than females.
Study on Cause of Death in Rat Neonates with Congenital Osteochondrodysplasia(ocd/ocd) II. Functional Abnormalities in Central Nervous System and Respiratory Muscle
Authors: KIKUKAWA Keiichiro , KAMEI Takayuki , SUZUKI Katsushi 
Published: May 2008

Abstract The rat with congenital osteochondrodysplasia with systemic subcutaneous edema (ocd/ocd) dies shortly after birth. It has been considered that the cause of death would be disturbed air flow from nose to lung by morphological abnormalities in respiratory tract. Respiratory rhythm was studied in the isolated and perfused preparation of brain stem‐spinal cord‐thorax in the ocd/ocd and phenotypically normal (+/?). The response of the isolated and perfused preparation of thorax and diaphragm to the electrical stimulation was also studied. The spontaneous depolarizations of cervical ventral root C4 or C5 in the brain stem‐spinal cord‐thorax preparation were very low and infrequent in the ocd/ocd. The both tensions of the thorax and diaphragm preparations to the electrical stimulation were lower in the ocd/ocd than those in the +/?.Those results show that the cause of death in the ocd/ocd is not only morphological abnormalities but also functional defects in the respiratory system including the central nervous system and muscles.
Prenatal Exposure to Atomic Radiation and Brain Damage*
Authors: OTAKE Masanori , YOSHIMARU Hiroshi , SCHULL William J. 
Published: May 2008

Abstract Evaluation of data on the frequency of severe mental retardation among prenatally exposed survivors of the atomic bombing of Hiroshima and Nagasaki, and the performance of such survivors on intelligence tests and in school has shown the most striking effects on the developing brain of exposure to ionizing radiation to occur among those individuals exposed in the 8th through the 15th, and the 16th through the 25th week after fertilization. This is true under the earlier T65DR and the newer DS86 systems of dosimetry. Seizure data have also revealed a significant effect of radiation in the 8‐15 week period after fertilization.Although the observed frequency of severe mental retardation in the 8‐15 week period can be satisfactorily described by a linear no‐threshold model with both dosimetries, there is some suggestion of a threshold in the low dose region with the DS86 uterine absorbed doses but not with the T65DR fetal absorbed doses. However, the location and even the existence of a threshold in these weeks after fertilization is difficult to demonstrate statistically with the DS86 uterine absorbed doses. If the threshold is estimated using all 19 cases of mental retardation within these weeks, the lower bound of the confidence interval of the estimate includes zero, and thus a threshold cannot be convincingly established. However, when we exclude two probable nonradiation‐related cases of Down's syndrome, the results suggest a threshold with a lower bound in the 0.10‐0.20 Gy region.Both dosimetries indicate a threshold in the dose‐response function for mental retardation in the 16‐25 week period, probably in the 0.23‐0.70 Gy interval.The seizure data provide no persuasive evidence of a threshold in the 8‐15 week period after fertilization; the 95% lower bound of the estimate of the threshold includes zero. Finally, although the mean IQ scores, and the mean school performances in the low dose region are similar to the values in the control group, particularly so with doses under 0.10 Gy, evidence for a threshold is not compelling.
Intrauterine Repair of Cleft Lip in Mouse Fetuses
Authors: IGAWA Hiroharu H. , OHURA Takehiko , IWAO Fumiya , YAMAMOTO Yuuhei , FUJIOKA Hirotaka 
Published: May 2008

AbstractSince most fetuses with drug‐induced cleft lip have concurrent cleft palate, they die shortly after birth as a result of difficulty in suckling. We devised an experimental model, in which cleft lip unaccompanied by cleft palate was produced artificially, in order to permit long‐term follow‐up of intrauterine cleft lip repair surgery.Fifteen Jcl:ICR mice each on days 15, 16, 17 and 18 of gestation (the day when a vaginal plug was found = day 0 of gestation) were used. Each mouse was laparotomized by midline incision of the lower abdomen, and the fetus at the distal end or the fetus adjacent to the fetus at the distal end of the uterine horn was subjected to surgery. Using a surgical microscope, the uterine wall was incised, and the amnion and chorion were pierced to expose the snout of the fetus. After unilateral complete cleft lip was created, the wound was repaired with 10–0 nylon sutures. The snout was then replaced within the uterus, and the surgical wound in the uterine wall was closed. The uterine horn was then placed back into the peritoneal cavity, and the abdominal wound was closed. The dams were then returned to their individual cages, and the fetuses were delivered spontaneously.The live birth rate for surgically treated fetuses was 60% in animals treated on day 15 of gestation, 73% among those treated on day 16, 80% among those treated on day 17, and 60% among those treated on day 18. The offspring underwent the normal course of postnatal growth, with good jaw development and no cicatrization at the site of upper lip surgery either grossly or histologically.
Experimental Hydrocephalus in Suckling Hamster Induced by Myxovirus Infection
Authors: TAKANO Tomoyuki , OHNO Masaki , YAMANO Tsunekazu , SHIMADA Morimi 
Published: May 2008

Abstract This study was undertaken to elucidate the pathogenesis of the hydrocephalus and aqueductal stenosis induced by intracerebral mumps virus inoculation in suckling hamsters.Mild ventricular dilatation became apparent after 5 days of inoculation. Focal denuding of the ependymal layer and subsequent aqueductal stenosis were observed by 14 days after inoculation. The virus antigen was detected not only in the ependymal cells and choroid plexus, but also in some neurons in the cerebral cortex, hippocampus, midbrain and cerebellum. In the cerebral aqueduct, the orderly arrangement of the cilialy clusters was destroyed on the 5th day after inoculation. After 10 days, proliferation of GFAP positive cells was noticed around the cerebral aqueduct and subsequently caused aqueductal stenosis. In the advanced state of hydrocephalus, the cerebellum was displaced downward and showed an elongated, atrophic and sleevelike structure similar to the Arnold‐Chiari malformation. It was suggested that the extensive damage of the ependymal cilia may account for early ventricular dilatation, and subsequent aqueductal stenosis with glial proliferation is the main cause of the advanced hydrocephalus. It has not yet been determined whether the mumps virus can pass through the human placenta or not. If it can, however, our results strongly suggest that mumps virus infection in the human fetus will cause congenital hydrocephalus.
Reduced Fertility in Female Homozygotes for hgn (Male Hypogonadism) Selected by hgn‐Associated Hypoplastic Kidney
Authors: SUZUKI Hiroetsu , HAKAMATA Yoji , KAMEI Takayuki , KIKUKAWA Keiichiro , SUZUKI Katsushi 
Published: May 2008

AbstractThe male hypogonadism rat (hgn/hgn) shows a characteristic male sterility as a single autosomal recessive trait. Recently, the female homozygotes for hgn, assumed to be fertile, could be detected by a /jgji‐associated hypoplastic kidney (hpk/hpk). The present study was to investigate a possible influence of the hgn gene on female reproduction. The hgn/hgn females showed a significant growth retardation as compared with the phenotypically normal ones (+/?; +/hgn or +/+). The litter size at birth and number of implantation traces were significantly less in the hgn/hgn than in the +/? females. The hgn/hgn females became anestrous and infertile much earlier than the +/? did. Histologically, there were a few corpora lutea, some atretic follicles at different stages of maturation and abundant abnormal interstitial cells with pyknotic or karyorexic nuclei in the ovaries of hgn/hgn females that have been infertile. The birth rate expressed by per cent litter size at birth against number of implantation traces was comparable between the hgn/hgn and the +/? female, suggesting that the small litter size of hgn/hgn female could not be due to the embryonic death in utero. Nevertheless, the number of the tubal ova at estrous was comparable in the hgn/hgn and +/? females. Therefore, it was suggested that the half of ova or embryos may be lost during the period from the fertilization to the implantation. Histological appearances of the neonatal ovary in the hgn/hgn seemed hypoplastic. The number of cells including oocytes and interstitial cells, enzymatically separated from neonatal ovary, was significantly less in the hgn/hgn than in the +/hgn. These results suggest that the gene product(s) coded by normal allele of hgn gene(s) involves normal gonadal development in both sexes; the defect may lead testicular dysmorphology in the male and reduced fertility in the female.
Radiation‐induced Anomalies: Report of a Study Conducted in Chernobyl*
Authors: SATOW Yukio , LAZJUK Gennady , TCHAIJUNUSOVA Nailya , ROZENSON Raphail , TAKEICHI Nobuo , KIMURA Akiro , KATOH Osamu , KAWAISHI Kuniko 
Published: May 2008

AbstractAt the 34th Annual Meeting of the Japanese Teratology Society held in July 1994, the first author was asked by President Hiroshi HARA to report on the results of the international joint study on congenital anomalies that we were conducting in the former Soviet Union. It was only a short time ago that the study was begun and long‐term observation needs to be made hereafter, but we made an interim report on the results obtained so far. In this report we describe (1) the results of a joint study made with Director Gennady Lazjuk of the Institute for Hereditary Diseases in Minsk, Republic of Belarus, mainly with regard to congenital anomalies that have increased after the Chernobyl nuclear power plant accident, (2) the results obtained in a study made with Dr. Anatoly Vacilez, Chief, Department of Obstetrics and Gynecology, Gomel District Hospital in Belarus, a heavily contaminated area in Belarus, and (3) the congenital anomalies that can be considered to have occurred as a result of the approximately 570 nuclear tests made over a period of 40 years from 1949 to 1989 in Semipalatinsk, Republic of Kazkh.
Congenital Anomalies of the Elbow Joint: Clinical Features and Classification
Authors: OGINO Toshihiko , ISHII Seiichi , SUGIMOTO Yoshihiro 
Published: May 2008

AbstractIn order to examine the distribution of different types of congenital anomalies of the elbow joint and discuss their classification, a total of 94 patients, 96 congenital anomalies of the elbow joint, were analyzed. They were classified according to the elbow deformity itself as follows; radio‐ulnar synostosis in 59 cases (61%), radial head dislocation in 23 cases (24%), ankylosis of the elbow joint in eight cases (8%), severe flexion contracture of the elbow joint in three cases (3%), and complete or partial aplasia of the elbow joint in three cases (3%). On the other hand, congenital elbow deformities can be classified into three types according to the associated anomalies. In 78 patients, in which congenital elbow deformity appeared as an independent deformity, there were radio‐ulnar synostosis in 56 patients, radial head dislocation in 19 patients, ankylosis of the elbow joint in two patients, and hypoplasia of trocholea in one patient. In the 13 patients, which congenital elbow deformity appeared as a part of congenital upper limb anomalies, the underlying congenital anomalies of the upper limb were ulnar deficiency in eight patients, radial deficiency in two patients, transverse deficiency in two patients, and phocomelia in one patient. In five patients, elbow deformity appeared as a part of congenital syndromes. Classification according to associated anomalies seems to be more suitable in order to examine the real distribution of different types of congenital deformities of the elbow joint as a part of the upper limb.
Congenital Anomalies Induced by Toxoplasma Infection
Authors: YOKOTA Kazuko 
Published: May 2008

Abstract A comprehensive review regarding congenital toxoplasmosis, which is caused by transmission of Toxoplasma gondii from an infected mother to her fetus during pregnancy, is presented. In most cases in humans the route of infection is oral and by either of the two forms of Toxoplasma; cysts in the raw flesh of an infected animal and oocysts in the feces of an infected cat. The clinical manifestations in 9 cases of congenital toxoplasmosis encountered by the author are preserted. The findings of computed tomography (CT) and magnetic resonance imaging (MRI) in 3 of these cases are presented. Delayed migration and myelination are suggested to have occurred in 2 cases in which abnormal MRI findings were noted. These MRI findings reflected the clinical manifestations and may also have reflected the pathologic findings.Experimental studies of transplacental infection following oral inoculation of Toxoplasma oocyst were performed in 3 groups of mice, in which infection of the dams was acute, subchronic, or chronic. The incidence of congenital infection was markedly higher in the acute infection group than in the subchronic or chronic infection groups. The incidence of 19S‐Toxoplasma (T)‐IgM positivity was also higher in the acute infection group than in either of the other two groups. Women should avoid contact with materials that have potentially been contaminated with cat feces, because Toxoplasma in the form of oocyst evacuated in cat feces is very highly infectious.Congenital toxoplasmosis is often accompanied by severe sequelae involving the central nervous system, but treatment is not effective once the symptoms are manifested. Early diagnosis and early treatment, before symptoms are manifest, are therefore important for the prevention of congenital toxoplasmosis.