Literature DB >> 16829350

Monosomy 8 rescue gave cells with a normal karyotype in a mildly affected man with 46,XY,r(8) mosaicism.

Gyri Aasland Gradek1, Per Helge Kvistad, Gunnar Houge.   

Abstract

The psychomotor and somatic development from early childhood into adult life is described in a man with 46,XY,r(8)/46,XY mosaicism. The ring chromosome 8 appeared to be of normal length on G-banding, but terminal deletions on 8q and 8p were detected with FISH and CGH. By STR marker analysis the 8p deletion proved to be quite large, at least 6.74 Mb, while the 8q deletion was small, around 2.5 Mb. The haplotype analysis also demonstrated that the r(8) originated from a maternal chromosome 8, and that cells with normal male karyotype resulted from monosomy 8 rescue after loss of the ring 8, i.e. a mitotic duplication of the paternal chromosome 8. The patient has a mild phenotype with no malformations and mild mental retardation, also compared to other ring chromosome 8 patients. His clinical condition has remained stable for the last 20 years.

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Year:  2005        PMID: 16829350     DOI: 10.1016/j.ejmg.2005.08.004

Source DB:  PubMed          Journal:  Eur J Med Genet        ISSN: 1769-7212            Impact factor:   2.708


  4 in total

Review 1.  Mosaicism for structural non-centromeric autosomal rearrangements in disease-defined carriers: sex differences in the rearrangements profile and maternal age distributions.

Authors:  Natalia V Kovaleva; Philip D Cotter
Journal:  Mol Cytogenet       Date:  2017-05-19       Impact factor: 2.009

2.  46,XY,r(8)/45,XY,-8 Mosaicism as a Possible Mechanism of the Imprinted Birk-Barel Syndrome: A Case Study.

Authors:  Anna A Kashevarova; Tatyana V Nikitina; Larisa I Mikhailik; Elena O Belyaeva; Stanislav A Vasilyev; Mariya E Lopatkina; Dmitry A Fedotov; Elizaveta A Fonova; Aleksei A Zarubin; Aleksei A Sivtsev; Nikolay A Skryabin; Lyudmila P Nazarenko; Igor N Lebedev
Journal:  Genes (Basel)       Date:  2020-12-09       Impact factor: 4.096

Review 3.  The past, present, and future for constitutional ring chromosomes: A report of the international consortium for human ring chromosomes.

Authors:  Peining Li; Barbara Dupont; Qiping Hu; Marco Crimi; Yiping Shen; Igor Lebedev; Thomas Liehr
Journal:  HGG Adv       Date:  2022-09-10

4.  Complex biology of constitutional ring chromosomes structure and (in)stability revealed by somatic cell reprogramming.

Authors:  T V Nikitina; A A Kashevarova; M M Gridina; M E Lopatkina; A A Khabarova; Yu S Yakovleva; A G Menzorov; Yu A Minina; I E Pristyazhnyuk; S A Vasilyev; D A Fedotov; O L Serov; I N Lebedev
Journal:  Sci Rep       Date:  2021-02-22       Impact factor: 4.379

  4 in total

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