Literature DB >> 33496066

Clinical and molecular characterization of four patients with Robinow syndrome from different families.

Archana Rai1, Siddaramappa J Patil2, Priyanka Srivastava3, Kalpana Gaurishankar4, Shubha R Phadke1.   

Abstract

Robinow syndrome (RS) is a rare heterogeneous disorder characterized by short stature, short-limbs, craniofacial, oro-dental abnormalities, vertebral segmentation defects, and frequently genital hypoplasia. Both autosomal dominant and recessive patterns of inheritance are observed with many causative genes. Here, we present the phenotypes and genotypes of four children with RS from different Indian families. Sequence variants were identified in genes ROR2, DVL1, and DVL3. Our results expand the mutational spectrum of RS and we also highlight the radiological changes in the radius and ulna in patients with ROR2 sequence variants which are primarily characteristic for ROR2 related RS but have been reported in WNT5A related RS.
© 2021 Wiley Periodicals LLC.

Entities:  

Keywords:  DVL1; DVL3; Indian; ROR2; Robinow syndrome; sequence variations

Mesh:

Substances:

Year:  2021        PMID: 33496066     DOI: 10.1002/ajmg.a.62082

Source DB:  PubMed          Journal:  Am J Med Genet A        ISSN: 1552-4825            Impact factor:   2.802


  2 in total

Review 1.  A novel frameshift mutation of DVL1-induced Robinow syndrome: A case report and literature review.

Authors:  Ruolan Hu; Yu Qiu; Yifei Li; Jinrong Li
Journal:  Mol Genet Genomic Med       Date:  2022-02-09       Impact factor: 2.183

2.  Novel pathogenic variants and quantitative phenotypic analyses of Robinow syndrome: WNT signaling perturbation and phenotypic variability.

Authors:  Chaofan Zhang; Angad Jolly; Brian J Shayota; Juliana F Mazzeu; Haowei Du; Moez Dawood; Patricia Celestino Soper; Ariadne Ramalho de Lima; Bárbara Merfort Ferreira; Zeynep Coban-Akdemir; Janson White; Deborah Shears; Fraser Robert Thomson; Sarah Louise Douglas; Andrew Wainwright; Kathryn Bailey; Paul Wordsworth; Mike Oldridge; Tracy Lester; Alistair D Calder; Katja Dumic; Siddharth Banka; Dian Donnai; Shalini N Jhangiani; Lorraine Potocki; Wendy K Chung; Sara Mora; Hope Northrup; Myla Ashfaq; Jill A Rosenfeld; Kati Mason; Lynda C Pollack; Allyn McConkie-Rosell; Wei Kelly; Marie McDonald; Natalie S Hauser; Peter Leahy; Cynthia M Powell; Raquel Boy; Rachel Sayuri Honjo; Fernando Kok; Lucia R Martelli; Vicente Odone Filho; Donna M Muzny; Richard A Gibbs; Jennifer E Posey; Pengfei Liu; James R Lupski; V Reid Sutton; Claudia M B Carvalho
Journal:  HGG Adv       Date:  2021-12-03
  2 in total

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