Literature DB >> 3789012

New evidence for genetic heterogeneity of the Freeman-Sheldon syndrome.

J M Sánchez, C P Kaminker.   

Abstract

We report on two sibs (a boy and a girl) with the Freeman-Sheldon (FS) syndrome (also called cranio-carpo-tarsal "dysplasia" or whistling face syndrome), born to normal parents. This unusual finding may represent genetic heterogeneity, germinal mutation of an autosomal dominant gene, and an epistatic recessive gene that suppressed the expression of the abnormal gene in the father, his parents being first cousins. The last 2 mechanisms seem less probable.

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Year:  1986        PMID: 3789012     DOI: 10.1002/ajmg.1320250312

Source DB:  PubMed          Journal:  Am J Med Genet        ISSN: 0148-7299


  2 in total

1.  Neonatal Schwartz-Jampel syndrome: a common autosomal recessive syndrome in the United Arab Emirates.

Authors:  L I Al-Gazali; M Varghese; E Varady; J Al Talabani; J Scorer; D Bakalinova
Journal:  J Med Genet       Date:  1996-03       Impact factor: 6.318

Review 2.  Freeman-sheldon syndrome presenting with microstomia: a case report and literature review.

Authors:  Vivek Gurjar; Anita Parushetti; Minal Gurjar
Journal:  J Maxillofac Oral Surg       Date:  2012-05-17
  2 in total

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