نتایج جستجو برای: spondylocostal dysplasia

تعداد نتایج: 28681  

Journal: :The Indian Journal of Pediatrics 2017

Journal: :Neuron 2003
Angelo Iulianella Kristin R Melton Paul A Trainor

Segmentation is a fundamental process in vertebrate embryogenesis, and one of the earliest manifestations of segmental patterning is the generation of transient, serially repeated blocks of mesodermal cells known as somites. Disruption of the normal segmentation process in humans leads to vertebral abnormalities such as spondylocostal dysostosis. In this minireview, we discuss recent advances i...

2016
Namita Chandra Sanjay Kumar Vaibhav Raj Pawan Kumar Vishwakarma Sheela Sinha Ram Prakash Saha

BACKGROUND Jarcho-Levin syndrome, also known as spondylothoracic dysplasia and spondylocostal dysplasia, is characterized by varieties of vertebrae and rib anomalies. Jarcho-Levin syndrome is a clinical-radiological diagnosis with clinical evidence of short neck, short trunk, normal-sized limbs, or increased arm span, and vertebral and rib defects on the skeletal survey. CASE REPORT About 400 c...

Journal: :Acta biochimica Polonica 2013
Bogdan Cylwik Karina Lipartowska Lech Chrostek Ewa Gruszewska

Glycosylation is a form of post-translational modification of proteins and occurs in every living cell. The carbohydrate chains attached to the proteins serve various functions. There are two main types of protein glycosylation: N-glycosylation and O-glycosylation. In this paper, we describe the O-glycosylation process and currently known congenital disorders of glycosylation associated with de...

Journal: :Japanese journal of human genetics 1987

2015
Cali E. Willet Mariano Makara George Reppas George Tsoukalas Richard Malik Bianca Haase Claire M. Wade

Spondylocostal dysostosis is a congenital disorder of the axial skeleton documented in human families from diverse racial backgrounds. The condition is characterised by truncal shortening, extensive hemivertebrae and rib anomalies including malalignment, fusion and reduction in number. Mutations in the Notch signalling pathway genes DLL3, MESP2, LFNG, HES7 and TBX6 have been associated with thi...

Journal: :Indian Journal of Physical Medicine and Rehabilitation 2018

Journal: :The American Journal of Human Genetics 2004

2015
Koichiro Abe Nobuhiko Takamatsu Kumiko Ishikawa Toshiko Tsurumi Sho Tanimoto Yukina Sakurai Thomas Lisse Kenji Imai Tadao Serikawa Tomoji Mashimo Tom J. Carney

Congenital vertebral malformations caused by embryonic segmentation defects are relatively common in humans and domestic animals. Although reverse genetics approaches in mice have provided information on the molecular mechanisms of embryonic somite segmentation, hypothesis-driven approaches cannot adequately reflect human dysmorphology within the population. In a N-ethyl-N-nitrosourea (ENU) mut...

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