| Literature DB >> 26333564 |
Sara Mumtaz1, Esra Yıldız2, Saliha Jabeen1, Amjad Khan1, Aslıhan Tolun2, Sajid Malik1.
Abstract
Primary microcephaly is clinically variable and genetically heterogeneous. Four phenotypically distinct types of autosomal recessive microcephaly syndromes are due to different RBBP8 mutations. We report on a consanguineous Pakistani family with homozygous RBBP8 mutation c.1808_1809delTA (p.Ile603Lysfs*7) manifesting microcephaly and a distinct combination of skeletal, limb and ectodermal defects, mild intellectual disability, minor facial anomalies, anonychia, disproportionate short stature and brachydactyly, and additionally talipes in one patient.Entities:
Keywords: Pakistani family; RBBP8; anonychia; consanguineous; inbreeding coefficient; intellectual disability; mental retardation; microcephaly; mutation; short stature
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Year: 2015 PMID: 26333564 DOI: 10.1002/ajmg.a.37299
Source DB: PubMed Journal: Am J Med Genet A ISSN: 1552-4825 Impact factor: 2.802