TGF-beta mediated Msx2 expression controls occipital somites-derived caudal region of skull development

Craniofacial development involves cranial neural crest (CNC) and mesoderm-derived cells. TGF-beta signaling plays a critical role in instructing CNC cells to form the craniofacial skeleton. However, it is not known how TGF-beta signaling regulates the fate of mesoderm-derived cells during craniofacial development. In this study, we show that occipital somites contribute to the caudal region of mammalian skull development. Conditional inactivation of Tgfbr2 in mesoderm-derived cells results in defects of the supraoccipital bone with meningoencephalocele and discontinuity of the neural arch of the C1 vertebra. At the cellular level, loss of TGF-beta signaling causes decreased chondrocyte proliferation and premature differentiation of cartilage to bone. Expression of Msx2, a critical factor in the formation of the dorsoventral axis, is diminished in the Tgfbr2 mutant. Significantly, overexpression of Msx2 in Myf5-Cre;Tgfbr2flox/flox mice partially rescues supraoccipital bone development. These results suggest that the TGF-beta/Msx2 signaling cascade is critical for development of the caudal region of the skull.     

Molecular diagnosis of Menkes disease: Genotype–phenotype correlation

Menkes syndrome is an X-linked, fatal neurodegenerative disorder of copper metabolism, caused by mutations in the ATP7A gene, encoding a copper-transporting P1B-type ATPase. To date, a total of approximately 160 different mutations have been reported worldwide. The clinical phenotypes observed in these patients include progressive neuro-degeneration, connective-tissue abnormalities and peculiar hair. There is phenotypic variability. While the majority of the patients do not survive early childhood, milder cases leading to longer survival have been reported. In this review we focus on mutations, identified in patients with milder forms of Menkes disease, and discuss the possibility of establishing a genotype–phenotype correlation. The presence of small amounts of normal protein, or the presence of partly functional protein variants containing a less essential amino acid substitution or a truncation of the N- or C-terminus, might all result in a milder, atypical phenotype. A clear phenotype–genotype correlation is however difficult to establish, clearly illustrated by the presence of inter- and even intra-familial variability.     

Event related desynchronization: use as a neurophysiologic marker is restricted

The present research aims to show that the occurrence of alpha blocking or event-related desynchronization (ERD) strongly depends on the amplitude and also on the phase angle of alpha activity at the stimulus onset. Simple visual stimulation was presented to 17 healthy subjects during EEG recording. An O₂ electrode was used for analysis with a 32 channel EEG sampling system. We used a segmentation of raw data in order to obtain the evoked potential. Prestimulus and poststimulus activities were filtered in the alpha (8–13 Hz) frequency band. Later, four different events (blocked, time-locked, phase-locked, and eliminated) were separately averaged. Phase-locked sweeps were determined by application of inter-trial coherence analysis. The evaluation of the data shows that “time-locked and phase-locked sweeps” were the dominating pattern and not “the blocked pattern”, which occurred only when the prestimulus alpha was high. In the analyses of EEG-EP sweeps, only 22 % of epochs showed (ERD). The ANOVA revealed significant differences between four different alpha responses (F(3,48) = 11.175; p < 0.001). Furthermore, alpha oscillations in time-locked responses were significantly higher than blocked (p < 0.0001). The analyses clearly demonstrate that important precaution is needed when using the ERD as a cognitive or pathological marker.     

Neurotensin Regulation of Endogenous Acetylcholine Release from Rat Cerebral Cortex: Effect of Quinolinic Acid Lesions of the Basal Forebrain

The effects of neurotensin (NT) on endogenous acetylcholine (ACh) release from basal forebrain, frontal cortex, and parietal cortex slices were tested. The results show that NT differentially regulates evoked ACh release from frontal and parietal cortex slices without altering either spontaneous or evoked ACh release from basal forebrain slices. In the frontal cortex, NT significantly inhibited evoked ACh release by a tetrodotoxin (TTX)-insensitive mechanism, suggesting an action directly on cholinergic terminals. In the parietal cortex, NT enhanced evoked ACh release by a TTX-sensitive mechanism, suggesting an action of NT on the cholinergic neuron or in close proximity to the cholinergic neuron. The effects of NT on ACh release were confined to evoked ACh release; that is, spontaneous ACh release was not affected. NT did not affect spontaneous or potassium-evoked ACh release from occipital cortex slices. The second set of experiments tested the effects of quinolinic acid (QUIN) lesions of the basal forebrain cell bodies on the NT-induced regulation of evoked ACh release in the cerebral cortex. QUIN lesions of basal forebrain cell bodies caused decreases in choline acetyltransferase activity (27 and 28%), spontaneous ACh release (14 and 21%), and evoked ACh release (38 and 44%) in frontal and parietal cortex, respectively. In addition, 11 days following QUIN lesions of basal forebrain cell bodies, the action of NT to regulate evoked ACh release in frontal cortex or parietal cortex was no longer observed. The results suggest that in the rat frontal and parietal cortex, NT differentially regulates the activity of cholinergic neurons by decreasing and increasing evoked ACh release, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)     

Fronto-cortical regulation of beta-endorphin actions in the rat

Ablation of the frontal neocortex markedly enhanced the antinociceptive and cataleptic actions of beta-endorphin injected into the lateral ventricle of rat brain. This enhanced response was not affected by simultaneous administration of cholecystokinin octapeptide (CCK-8). In sham-operated rats, however, CCK-8 suppressed the effects of beta-endorphin in a dose-related manner. Moreover, ablation of a similar amount of occipital neocortex did neither affect beta-endorphin actions nor the interactions of CCK-8.     

TGF-β mediated FGF10 signaling in cranial neural crest cells controls development of myogenic progenitor cells through tissue-tissue interactions during tongue morphogenesis

Skeletal muscles are formed from two cell lineages, myogenic and fibroblastic. Mesoderm-derived myogenic progenitors form muscle cells whereas fibroblastic cells give rise to the supportive connective tissue of skeletal muscles, such as the tendons and perimysium. It remains unknown how myogenic and fibroblastic cell-cell interactions affect cell fate determination and the organization of skeletal muscle. In the present study, we investigated the functional significance of cell-cell interactions in regulating skeletal muscle development. Our study shows that cranial neural crest (CNC) cells give rise to the fibroblastic cells of the tongue skeletal muscle in mice. Loss of Tgfbr2 in CNC cells (Wnt1-Cre;Tgfbr2^flox/flox) results in microglossia with reduced Scleraxis and Fgf10 expression as well as decreased myogenic cell proliferation, reduced cell number and disorganized tongue muscles. Furthermore, TGF-β2 beads induced the expression of Scleraxis in tongue explant cultures. The addition of FGF10 rescued the muscle cell number in Wnt1-Cre;Tgfbr2^flox/flox mice. Thus, TGF-β induced FGF10 signaling has a critical function in regulating tissue-tissue interaction during tongue skeletal muscle development.     

枕骨圆枕的变异

对印第安人,阿留申人,华北人和云南人总共５０７具头骨的枕骨圆枕发育程度进行观察,结果表明枕骨圆枕任一发育级别的出现率在相同人种且性别相同的人群之间并无显著差别,枕骨圆枕的发育程度可以被认为是“遗传标记”。作者认为在化石人类中枕骨圆枕的发育程度会有较大的变异;枕骨圆枕的发育程度不是一个有效的鉴别性状。     

Migration of cranial neural crest cells to the pharyngeal arches and heart in rat embryos

Summary The existence of a neural crest cell migration pathway from occipital levels of the hindbrain into the heart was suspected in mammalian embryos because it had previously been identified in avian embryos and because the Di George anomaly, an association between craniofacial and cardiac malformations, is most easily explained on the basis of abnormal neural crest cell migration to all of the affected structures. In order to demonstrate the existence of this pathway, neural crest cells were labelled in situ in rat embryos with the fluorescent dye DiI, and the embryos cultured for up to 48 h. Cells labelled between occipital somites 1 and 2 or 3 and 4 migrated within and dorsal to the third and fourth pharyngeal arches and into the outflow tract of the heart (conus cordis and truncus arteriosus). The cardiac labelling was in individually visible cells, in contrast to the mass of fluorescence seen in the pharyngeal and dorsal mesenchyme. Within the outflow tract wall, the labelled cells were enmeshed by strands of alcian blue-stained extracellular matrix. There was no labelling of cardiac cells following injections just rostral to, or just caudal to, somites one and four. This study establishes the existence and precise levels of origin of the cardiac neural crest in a mammalian embryo.     

Response properties of auditory activated cells in the occipital cortex of the blind mole rat: an electrophysiological study

Previous studies have demonstrated that despite its blindness, the subterranean blind mole rat (Spalax ehrenbergi) possesses a noticeable lateral geniculate nucleus and a typical cyto-architectural occipital cortex that are reciprocally connected. These two areas, as revealed by the metabolic tracer 2-deoxyglucose, are activated by auditory stimuli. Using single unit recordings, we show that about 57% of 325 cells located within the occipital cortex of anesthetized mole rats responded to at least one of the following auditory stimuli — white noise, pure tones, clicks, and amplitude modulated tones — with the latter two being the most effective. About 85% of cells driven by either contralateral or ipsilateral stimulation also responded to binaural stimulation; about 13% responded only to binaural stimulation; and 2% were driven exclusively by contralateral stimulation. Comparing responsiveness and response strength to these three modes of stimulation revealed a contralateral predominance. Mean latency (±SD) of ipsilateral and contralateral responses were 48.5±32.6 ms and 33.5±9.4 ms, respectively. Characteristic frequencies could be divided into two distinct subgroups ranging between 80 and 125 Hz and between 2,500 and 4,400 Hz, corresponding to the most intensive spectral components of the vibratory intraspecific communication signals and airborne vocalizations.Abbreviations BMF best modulation frequency - CF characteristic frequency - 2-DG 2-deoxyglucose - dLGN dorsal lateral geniculate nucleus - IC inferior colliculus - LGN lateral geniculate nucleus - OC occipital cortex - MTF modulation transfer function - SAM sinusoidally amplitude modulation - SC superior colliculus     

青岛出土颅骨某些径的测量

对国人颅骨径的测量已不少见,但多为依据骨骼人类学性别特征进行性别鉴定、或未经性别鉴定的材料,而对已知生前性别者的测量并不甚多,为了积累国人体质材料,并为人类学、解剖学及法医学等研究提供参考依据,我们按《人体骨骼测量方法》（吴汝康等,1965）和《人体测量手册》（邵象清,1985）等所列标准为主要依据,对青岛出土、已知性别（据墓碑记载）、多为汉族、较完整的312例（男154,女158）成人颅骨进行了十四项径的直线测量,对所测数据进行了统计学处理和性别差异检验。现将主要结果列于上页表。