Yayınlanmış 1 Ocak 2014 | Sürüm v1
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Loss-of-Function Mutations in PNPLA6 Encoding Neuropathy Target Esterase Underlie Pubertal Failure and Neurological Deficits in Gordon Holmes Syndrome

  • 1. Oregon Natl Primate Res Ctr, Div Neurosci, Beaverton, OR 97006 USA
  • 2. Oregon Hlth & Sci Univ, Oregon Inst Occupat Hlth Sci, Portland, OR 97239 USA
  • 3. Cukurova Univ, Inst Sci, Dept Biotechnol, TR-01330 Adana, Turkey
  • 4. Univ Bristol, Sch Clin Sci, Labs Integrat Neurosci & Endocrinol, Bristol BS1 3NY, Avon, England
  • 5. Cukurova Univ, Fac Med, Dept Neurol, TR-01330 Adana, Turkey
  • 6. Radboud Univ Nijmegen, Nijmegen Med Ctr, Dept Human Genet, NL-6525 GA Nijmegen, Netherlands
  • 7. Uludag Univ, Sch Med, Dept Endocrinol & Metab, TR-16110 Bursa, Turkey
  • 8. Uludag Univ, Sch Med, Dept Pediat Endocrinol & Metab, TR-16110 Bursa, Turkey
  • 9. Cukurova Univ, Fac Med, Div Pediat Endocrinol, TR-01330 Adana, Turkey
  • 10. Univ Cordoba, Dept Cell Biol Physiol & Immunol, E-14071 Cordoba, Spain

Açıklama

Context: Gordon Holmes syndrome (GHS) is characterized by cerebellar ataxia/atrophy and normosmic hypogonadotropic hypogonadism (nHH). The underlying pathophysiology of this combined neurodegeneration and nHH remains unknown.

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