Peters Plus syndrome is caused by mutations in B3GALTL, a putative glycosyltransferase

Saskia A. J. Lesnik Oberstein, Marjolein Kriek, Stefan J. White, Margot E. Kalf, Karoly Szuhai, Johan T. den Dunnen, Martijn H. Breuning, Raoul C. M. Hennekam

Research output: Contribution to journalArticleAcademicpeer-review

161 Citations (Scopus)

Abstract

Peters Plus syndrome is an autosomal recessive disorder characterized by anterior eye-chamber abnormalities, disproportionate short stature, and developmental delay. After detection of a microdeletion by array-based comparative genomic hybridization, we identified biallelic truncating mutations in the beta 1,3-galactosyltransferase-like gene (B3GALTL) in all 20 tested patients, showing that Peters Plus is a monogenic, primarily single-mutation syndrome. This finding is expected to put Peters Plus syndrome on the growing list of congenital malformation syndromes caused by glycosylation defects
Original languageEnglish
Pages (from-to)562-566
JournalAmerican journal of human genetics
Volume79
Issue number3
DOIs
Publication statusPublished - 2006

Cite this