Marisol Ibarra-Ramírez, Luis Daniel Campos-Acevedo, Laura E Martínez de Villarreal
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引用次数: 0
摘要
特纳综合征(Turner Syndrome,TS)是由第二条性染色体全部或部分缺失引起的,每 2,500-3,000 名活产婴儿中就有一名特纳综合征患者。临床表型变化很大,包括身材矮小和性腺发育不良。1959 年,该综合征的染色体来源得到确认;患者有 45 条染色体,其中只有一条 X 染色体。TS 表现为性染色体数目和结构异常,有趣的是,只有 40% 的患者具有 45 X 染色体核型。其余的染色体异常包括镶嵌、X 染色体短臂和长臂缺失、环和异染色体。尽管有多项研究旨在确定 TS 的临床特征与不同染色体变异之间的关系,但目前还无法确定两者之间的明确关联。目前,人们已对表型所涉及的不同机制进行了探讨。本综述将重点分析 TS 的不同染色体异常和表型,并讨论导致这些异常的可能机制。
Chromosomal Abnormalities of Interest in Turner Syndrome: An Update.
Turner syndrome (TS) is caused by the total or partial loss of the second sex chromosome; it occurs in 1 every 2,500-3,000 live births. The clinical phenotype is highly variable and includes short stature and gonadal dysgenesis. In 1959, the chromosomal origin of the syndrome was recognized; patients had 45 chromosomes with a single X chromosome. TS presents numerical and structural abnormalities in the sex chromosomes, interestingly only 40% have a 45, X karyotype. The rest of the chromosomal abnormalities include mosaics, deletions of the short and long arms of the X chromosome, rings, and isochromosomes. Despite multiple studies to establish a relationship between the clinical characteristics and the different chromosomal variants in TS, a clear association cannot yet be established. Currently, different mechanisms involved in the phenotype have been explored. This review focuses to analyze the different chromosomal abnormalities and phenotypes in TS and discusses the possible mechanisms that lead to these abnormalities.
期刊介绍:
Neuroscience and Behavioral Physiology publishes contributions of international importance in the fields of physiology, psychopharmacology, neuroendocrinology and neurochemistry, as well as in the anatomy and pathology of the nervous system. Special attention is given to research relating to brain function with behavior.
Neuroscience and Behavioral Physiology also publishes translations of papers selected from top Russian journals describing significant results of studies on the nervous system. Priority is given to new experimental findings.
Reviews are also provided of the current status of entire fields of Russian research.
Brings results of top research into nervous system from multidisciplinary perspective
Gives priority of the new experimental data
Focuses on research findings relating brain function and behavior
100% of authors who answered a survey reported that they would definitely publish or probably publish in the journal again