American journal of medical genetics. Part A · 2005
Acro-dermato-ungual-lacrimal-tooth (ADULT) syndrome: report of a child with phenotypic overlap with ulnar-mammary syndrome and a new mutation in TP63.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Slavotinek Anne M, Tanaka June, Winder Alison, Vargervik Karin, Haggstrom Anita, Bamshad Michael
Аннотация
We report on a new patient with clinical findings consistent with acro-dermato-ungual-lacrimal-tooth (ADULT) syndrome. The child had sparse hair, extensive freckling, lacrimal duct stenosis, oligodontia, dystrophic nails, reduced sweating, and bilateral athelia. Examination of his hands showed ulnar ray hypoplasia with bilateral fifth finger brachydactyly and camptodactyly. He also had surgical repair of an imperforate anus. Mutation analysis of TP63 showed a single nucleotide substitution, c.G518A, predicting a novel missense mutation, p.V114M in exon 4. This is the third mutation to be reported in TP63 in ADULT syndrome.
Предметные рубрики MeSH
Abnormalities, MultipleBase SequenceDNA Mutational AnalysisDNA-Binding ProteinsDiagnosis, DifferentialEctodermal DysplasiaFemaleFingersGenes, Tumor SuppressorHumansLacrimal Duct ObstructionMaleMammary Glands, HumanMutation, MissensePedigreePhenotypePhosphoproteinsSyndromeTooth AbnormalitiesTrans-ActivatorsTranscription FactorsTumor Suppressor ProteinsUlna