Abnormalities May Be Related Defective Somitogenesis: Multiple Vertebla Segmentation Defects And Neural Tube Defects

E. Odacı; A. Yıldıran; Ş. Küçüködük

doi:10.5835/jecm.v18i3.291

Abnormalities May Be Related Defective Somitogenesis: Multiple Vertebla Segmentation Defects And Neural Tube Defects

Year 2001, Volume: 18 Issue: 3, - , 23.12.2009

Abstract

Multiple vertebral segmentation defects (MVSD) (MIM#277300) including hemi vertebrae, fused or absent ribs, and "crab-like" thorax has been reported under various names such as spondylothoracic dysplasia, spondylocostal dysostosis, Jarcho-Levin Syndrome and others. Many different genetic mutations reported in order to explain these defects. But the manifestation of MVSD are various and complex, so these mutation couldn't be satisfactory. Then, how can it be explained this maldevelopment? As a consequence of their segmented arrangement and the diversity of their tissue derivatives, somites are key elements in the establishment of the metameric body plan in vertebrates; probably, disregulation of somite differentiation and proliferation may disclose the problem. We conclude that defective somitogenesis should be investigated carefully to explain related clinical abnormalities.

Defektif Somitogenesis ile İlişkili Anomaliler: Multipl Vertebral Segmetasyon ve Nöral Tüp Defektleri

Hemivertebra, kosta füzyonu .veya yokluğu, yengeçvari toraks gibi anomalilerle görülebilen Multipl Vertebral Segmetasyon DefekÜeri (MVSD) (MIM#277300) literatürde spondilotorasik displazi, spondilokostal disostoz, Jarcho-Levin Sendromu gibi çeşitli isimlerle bildirilmiştir. Bu defektleri açıklamak için pek çok değişik genetik mutasyon ileri sürülmüştür. Fakat, MVSD'nin manifestasyonları o kadar çok ve değişiktir ki bu mu-tasyonlar bütün bulgulan açıklamamaktadır. Bu durumda bu gelişim bozuklukları nasıl açıklanabilir? Somitler segmente yapılan ve geliştirdikleri dokular bakımından ver-tebralılann metamerik vücut yapılarını açıklamakta anahtar durumundadırlar. Muhtemelen somit diferansiyasyonu ve proliferasyonundaki bozukluklar oluş mekanizmasını açıklamakta önem kazanmaktadır. İlgili anomalilerin açıklanabilmesi için defektif so-mitogenezin araştmlması gerektiğini düşünüyoruz.

Keywords

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Year 2001, Volume: 18 Issue: 3, - , 23.12.2009

E. Odacı A. Yıldıran Ş. Küçüködük

Abstract

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Details

Primary Language	English
Journal Section	Basic Medical Sciences
Authors	E. Odacı This is me A. Yıldıran This is me Ş. Küçüködük This is me
Publication Date	December 23, 2009
Submission Date	November 16, 2009
Published in Issue	Year 2001 Volume: 18 Issue: 3

Cite

APA	Odacı, E., Yıldıran, A., & Küçüködük, Ş. (2009). Abnormalities May Be Related Defective Somitogenesis: Multiple Vertebla Segmentation Defects And Neural Tube Defects. Journal of Experimental and Clinical Medicine, 18(3). https://doi.org/10.5835/jecm.v18i3.291
AMA	Odacı E, Yıldıran A, Küçüködük Ş. Abnormalities May Be Related Defective Somitogenesis: Multiple Vertebla Segmentation Defects And Neural Tube Defects. J. Exp. Clin. Med. December 2009;18(3). doi:10.5835/jecm.v18i3.291
Chicago	Odacı, E., A. Yıldıran, and Ş. Küçüködük. “Abnormalities May Be Related Defective Somitogenesis: Multiple Vertebla Segmentation Defects And Neural Tube Defects”. Journal of Experimental and Clinical Medicine 18, no. 3 (December 2009). https://doi.org/10.5835/jecm.v18i3.291.
EndNote	Odacı E, Yıldıran A, Küçüködük Ş (December 1, 2009) Abnormalities May Be Related Defective Somitogenesis: Multiple Vertebla Segmentation Defects And Neural Tube Defects. Journal of Experimental and Clinical Medicine 18 3
IEEE	E. Odacı, A. Yıldıran, and Ş. Küçüködük, “Abnormalities May Be Related Defective Somitogenesis: Multiple Vertebla Segmentation Defects And Neural Tube Defects”, J. Exp. Clin. Med., vol. 18, no. 3, 2009, doi: 10.5835/jecm.v18i3.291.
ISNAD	Odacı, E. et al. “Abnormalities May Be Related Defective Somitogenesis: Multiple Vertebla Segmentation Defects And Neural Tube Defects”. Journal of Experimental and Clinical Medicine 18/3 (December 2009). https://doi.org/10.5835/jecm.v18i3.291.
JAMA	Odacı E, Yıldıran A, Küçüködük Ş. Abnormalities May Be Related Defective Somitogenesis: Multiple Vertebla Segmentation Defects And Neural Tube Defects. J. Exp. Clin. Med. 2009;18. doi:10.5835/jecm.v18i3.291.
MLA	Odacı, E. et al. “Abnormalities May Be Related Defective Somitogenesis: Multiple Vertebla Segmentation Defects And Neural Tube Defects”. Journal of Experimental and Clinical Medicine, vol. 18, no. 3, 2009, doi:10.5835/jecm.v18i3.291.
Vancouver	Odacı E, Yıldıran A, Küçüködük Ş. Abnormalities May Be Related Defective Somitogenesis: Multiple Vertebla Segmentation Defects And Neural Tube Defects. J. Exp. Clin. Med. 2009;18(3).