• Toxicological Research
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• 제17권
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• pp.11-16
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• 2001

The development of the central nervous system is a continuous process during the embryonic and fetal periods. For a better understanding of congenital anomalies of central nervous system, three major events of normal development, i.e., neurulation (3 to 4 weeks), brain vesicle formation (4 to 7 weeks) and mantle formation (over 8 weeks) should be kept in mind. The first category of anomalies is neural tube defect. Neural tube defects encompass all the anomalies arise in completion of neurulation. The second category of central nervous system anomalies is disorders of brain vesicle formation. This is anomaly that applies for "the face predicts the brain". Holoprosencephaly covers a spectrum of anomalies of intracranial and midfacial development which result from incomplete development and septation of midline structures within the forebrain or prosencephalon. The last category of central nervous system malformation is disorders involving the process of mantle formation. In the human, neurons are generated in two bursts, the first from 8 to 10 weeks and next from 12 to 14 weeks. By 16 weeks, most of the neurons have been generated and have started their migration into the cortex. Mechanism of migration disorders are multifactorial. Abnormal migration into the cortex, abnormal neurons, faulty neural growth within the cortex, unstable pial-glial border, degeneration of neurons, neural death by exogenous factors are some of the proposed mechanism. Agyria-pachygyria are characterized by a four-layerd cortex. Polymicrogyria is gyri that are too numerous and too small, and is morphologically heterogeneous. Cortical dysplasia is characterized by the presence Q[ abnormal neurons and glia arranged abnormally in focal areas of the cerebral cortex. Neuroglial malformative lesions associated with medically intractable epilepsy are hamartia or hamartoma, focal cortical dysplasia and microdysgenesis.ysgenesis.

• Journal of Korean Neurosurgical Society
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• 제62권3호
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• pp.265-271
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• 2019

The expansion and folding of the cerebral cortex occur during brain development and are critical factors that influence cognitive ability and sensorimotor skills. The disruption of cortical growth and folding may cause neurological disorders, resulting in severe intellectual disability and intractable epilepsy in humans. Therefore, understanding the mechanism that regulates cortical growth and folding will be crucial in deciphering the key steps of brain development and finding new therapeutic targets for the congenital anomalies of the cerebral cortex. This review will start with a brief introduction describing the anatomy of the brain cortex, followed by a description of our understanding of the proliferation, differentiation, and migration of neural progenitors and important genes and molecules that are involved in these processes. Finally, various types of disorders that develop due to malformation of the cerebral cortex will be discussed.

• Investigative Magnetic Resonance Imaging
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• 제3권1호
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• pp.60-65
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• 1999

목적 : 뇌백질 신호억제를 위한 중간시간 반전회복(Medium Tau Inversion Recovery, MTIR)영상에서 뇌회질과 뇌배질의 대조도를 다른 기법의 MR영상과 비교해 보고 뇌피질에 이상이 있는 환자에서 MTIR영상의 유용성을 평가하고자 하였다. 대상 및 방법 : 2명의 정상 지원자와 뇌피질 이형성증을 포함한 뇌피질 질환이 있는 21명을 대상으로 뇌회질과 뇌백질의 신호의 차이를 관심영역에서 대조도 백분율과 대조도 잡음비로 츠정하여 MTIR영상과 여러 가지 다른 MR영상을 비교하였다. 또한 시각적으로 병변이 뚜렷함, 새로운 병변의 발견여부를 시각적으로 비교 평가하였다. 결과 : MTIR영상은 다른 MR영상에 비해 대조도 백분율, 대조도 잡음비가 높아 뇌회질과 뇌백질의 신호의 차이가 가장 뚜렷하였다. 신경이주이상을 포함한 21명의 뇌피질 환자에서는 MTIR영상에서 다른 영상보다 병변이 뚜려사고 병변의 묘사(delineation)을 증가 시켰으나 새로운 병변은 발견하지 못해다. 결론 : MTIR영상은 뇌회질과 뇌백질의 대조도를 증가시키는 영상 기법이며 뇌피질을 침범한 질환을 특히 뇌피질 이형성증의 병변을 매우 잘 나타냈다. 기존의 T1강조영상 또는 3D-MPRAG에서 뇌피질-백질의 구별이 어려운 경우에는 보완적으로 이용가치가 있는 영상기법으로 생각된다.

• International Journal of Molecular Medicine
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• 제43권2호
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• pp.1105-1113
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• 2019

Epigenetic modifier lysine demethylase 3a (Kdm3a) specifically demethylates mono- and di-methylated ninth lysine of histone 3 and belongs to the Jumonji domain-containing group of demethylases. Kdm3a serves roles during various biological and pathophysiological processes, including spermatogenesis and metabolism, determination of sex, androgen receptor-mediated transcription and embryonic carcinoma cell differentiation. In the present study, physiological functions of Kdm3a were evaluated during embryogenesis of Xenopus laevis. Spatiotemporal expression pattern indicated that kdm3a exhibited its expression from early embryonic stages until tadpole stage, however considerable increase of kdm3a expression was observed during the neurula stage of Xenopus development. Depleting kdm3a using kdm3a antisense morpholino oligonucleotides induced anomalies, including head deformities, small-sized eyes and abnormal pigmentation. Whole-mount in situ hybridization results demonstrated that kdm3a knockdown was associated with defects in neural crest migration. Further, quantitative polymerase chain reaction revealed abnormal expression of neural markers in kdm3a morphants. RNA sequencing of kdm3a morphants indicated that kdm3a was implicated in mesoderm formation, cell adhesion and metabolic processes of embryonic development. In conclusion, the results of the present study indicated that Kdm3a may serve a role in neural development during Xenopus embryogenesis and may be targeted for treatment of developmental disorders. Further investigation is required to elucidate the molecular mechanism underlying the regulation of neural development by Kdm3a.