Literature DB >> 26850509

Pre- and post-natal growth in two sisters with 3-M syndrome.

Licia Lugli1, Emma Bertucci2, Vincenzo Mazza2, Amira Elmakky3, Fabrizio Ferrari1, Christine Neuhaus4, Antonio Percesepe5.   

Abstract

3-M syndrome (OMIM #273750) is a rare autosomal recessive growth disorder characterized by severe pre- and post-natal growth restriction, associated with minor skeletal abnormalities and dysmorphisms. Although the 3-M syndrome is well known as a primordial dwarfism, descriptions of the prenatal growth are missing. We report a family with variable phenotypic features of 3-M syndrome and we describe the prenatal and postnatal growth pattern of two affected sisters with a novel homozygous CUL7 mutation (c.3173-1G>C), showing a pre- and post-natal growth deficiency and a normal cranial circumference.
Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Entities:  

Keywords:  3-M syndrome; CUL7; Dwarfism; Knee dislocations

Mesh:

Substances:

Year:  2016        PMID: 26850509     DOI: 10.1016/j.ejmg.2016.01.009

Source DB:  PubMed          Journal:  Eur J Med Genet        ISSN: 1769-7212            Impact factor:   2.708


  5 in total

1.  Spectrum of X-linked intellectual disabilities and psychiatric symptoms in a family harbouring a Xp22.12 microduplication encompassing the RPS6KA3 gene.

Authors:  Vera Uliana; Francesco Bonatti; Valentina Zanatta; Paola Mozzoni; Davide Martorana; Antonio Percesepe
Journal:  J Genet       Date:  2019-03       Impact factor: 1.166

Review 2.  Nonclassical GH Insensitivity: Characterization of Mild Abnormalities of GH Action.

Authors:  Helen L Storr; Sumana Chatterjee; Louise A Metherell; Corinne Foley; Ron G Rosenfeld; Philippe F Backeljauw; Andrew Dauber; Martin O Savage; Vivian Hwa
Journal:  Endocr Rev       Date:  2019-04-01       Impact factor: 19.871

Review 3.  Cullin-RING E3 Ubiquitin Ligase 7 in Growth Control and Cancer.

Authors:  Zhen-Qiang Pan
Journal:  Adv Exp Med Biol       Date:  2020       Impact factor: 2.622

4.  Identification of two CUL7 variants in two Chinese families with 3-M syndrome by whole-exome sequencing.

Authors:  Li Hu; Xike Wang; Tingting Jin; Yuanyuan Han; Juan Liu; Minmin Jiang; Shujuan Yan; Xiaoling Fu; Bangquan An; Shengwen Huang
Journal:  J Clin Lab Anal       Date:  2020-03-06       Impact factor: 2.352

5.  Novel CUL7 biallelic mutations alter the skeletal phenotype of 3M syndrome.

Authors:  Nao Takizaki; Yoshinori Tsurusaki; Kaoru Katsumata; Yumi Enomoto; Hiroaki Murakami; Koji Muroya; Hiroshi Ishikawa; Noriko Aida; Gen Nishimura; Kenji Kurosawa
Journal:  Hum Genome Var       Date:  2020-02-04
  5 in total

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