Dental developmental abnormalities in a patient with subtelomeric 7q36 deletion syndrome may confirm a novel role for the SHH gene

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Linhares, Natália D.; Svartman, Marta; Salgado, Mauro Ivan; Rodrigues, Tatiane C.; da Costa, Silvia S.; Rosenberg, Carla; Valadares, Eugênia R.;
  • Publisher: Elsevier BV
  • Journal: Meta Gene,volume 2,pages16-24 (issn: 2214-5400, eissn: 2214-5400)
  • Publisher copyright policies & self-archiving
  • Related identifiers: pmc: PMC4287791, doi: 10.1016/j.mgene.2013.10.005
  • Subject: 7q deletion | Human HTR5A protein | OFC, occipitofrontal circumference | Short Communication | Human MNX1 protein | Comparative genomic hybridization | ASD, autism spectrum disorder | BERA, brainstem evoked response audiometry | CNV, copy number variation | Human EN2 protein | Human SHH protein | aCGH, array comparative genomic hybridization | FISH, fluorescence in situ hybridization
    mesheuropmc: stomatognathic diseases | stomatognathic system

Studies in mice demonstrated that the Shh gene is crucial for normal development of both incisors and molars, causing a severe retardation in tooth growth, which leads to abnormal placement of the tooth in the jaw and disrupted tooth morphogenesis. In humans the SHH gen... View more
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