A small de novo 16q24.1 duplication in a woman with severe clinical features.

نویسندگان

  • Sylvia Quéméner-Redon
  • Caroline Bénech
  • Séverine Audebert-Bellanger
  • Gaëlle Friocourt
  • Marc Planes
  • Philippe Parent
  • Claude Férec
چکیده

We report here a de novo 16q24.1 interstitial duplication in a woman with a severe phenotype consistent with mental retardation, spastic paraplegia, severe epilepsy, a narrow and arched palate, malar hypoplasia, little subcutaneous fat and arachnodactyly. Although conventional karyotyping was found to be normal, array-CGH detected a small duplication on chromosome 16. Using QFM-PCR, we characterised its proximal and distal breakpoints. The duplication, which is approximately 250 kb, encompasses seven genes (KIAA0182, GINS2, c16orf74, COX4NB, COX4I1, MIR1910 and IRF8). Several reports have previously described large 16q duplications, and some of these overlap with our region in 16q24.1. Due to the variability of the described phenotypes, the characterisation of small 16q duplications may help to determine critical regions and the genes they contain that are associated with the components of complex phenotypes.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Partial Distal 10q Trisomy Due to De Novo Amplification: A new Case Without Furrows or Ridges in Fingers and Palms

Background: Here we describe a new case of partial distal 10q trisomy in a 6-year-old Iranian girl from healthy parents with mental, growth, and psychomotor retardations. Methods: Additional clinical features include dysmorphic craniofacial features, microcephaly, bilateral hydronephrosis without heart problems, small and rotated low-set ears, bow-shaped mouth, abnormal teeth, short neck, an...

متن کامل

AB184. Effects of target gene expression on ex vivo differentiated mesenchymal stromal cells transfected by lent viral vector with PDE5 of short hairpin RNA

Background: Partial trisomy 16q is a rare syndrome with a wide range of manifestations. We here reported a boy patient with an inherited balanced complex chromosome rearrangement (CCR) involving chromosomes 1, 5, 12 and 13, and a de novo pure partial trisomy 16q24.1-24.3. Methods: We performed G-banding analysis of the peripheral blood, chromosomal microarray analysis (CytoScanTM HD Array) and ...

متن کامل

A Rare Case of Duplication of Chromosome 2 (q31.3q36.3) in a 4.5-year-old Boy and Review of the Literature

De novo duplication of 2q is very rare. Most cases of 2q duplications result from familial translocations, and are associated with simultaneous monosomy of another chromosome segment. To our knowledge and search in English literature there are less than 20 reported cases of isolated 2q duplication. Hereby we introduce a 4.5-year-old Iranian boy of a non-consanguineous marriage who was referred ...

متن کامل

The First Case of a Small Supernumerary Marker Chromosome 18 in a Klinefelter Fetus: A Case Report

Small supernumerary marker chromosomes (sSMCs), or markers, are abnormal chromosomal fragments that can be hereditary or de novo. Despite the importance of sSMCs diagnosis, de novo sSMCs are rarely detected during the prenatal diagnosis process. Usually, prenatally diagnosed de novo sSMCs cannot be correlated with a particular phenotype without knowing their chromosomal origin and content; ther...

متن کامل

Report of an interesting case of small bowel duplication [Persian]

  A 13 years old boy with severe abdominal pain and history of intermittent (II bleeding since the aye of 3 months, is presented. Different diagnostic procedures were unremarkable. A Tc-99m Pertechnetate abdominal study revealed abnormal radionuclide accumulation in small bowel region. Duplication of small bowel was suspected which was confirmed by surgery and patholo...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • European journal of medical genetics

دوره 56 4  شماره 

صفحات  -

تاریخ انتشار 2013