Literature DB >> 1677119

Hereditary unstable DNA: a new explanation for some old genetic questions?

G R Sutherland1, E A Haan, E Kremer, M Lynch, M Pritchard, S Yu, R I Richards.   

Abstract

Fragile X syndrome, associated with the fragile X chromosome, is the most common cause of familial mental retardation. The condition is characterised by a heritable DNA sequence that consists of an abnormal number of CCG repeats, and which is unstable in both mitosis and meiosis. We suggest that such heritable unstable DNA sequences could be present in other parts of the genome and that these might explain a number of genetic events that are not well understood in terms of classic genetic mechanisms. Such poorly explained observations include anticipation, incomplete penetrance, variable expression, and possibly imprinting, variegation, and multifactorial inheritance.

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Year:  1991        PMID: 1677119     DOI: 10.1016/0140-6736(91)90426-p

Source DB:  PubMed          Journal:  Lancet        ISSN: 0140-6736            Impact factor:   79.321


  21 in total

1.  Exclusion mapping of the X-linked dominant chondrodysplasia punctata/ichthyosis/cataract/short stature (Happle) syndrome: possible involvement of an unstable pre-mutation.

Authors:  H Traupe; D Müller; D Atherton; D C Kalter; F P Cremers; B A van Oost; H H Ropers
Journal:  Hum Genet       Date:  1992-08       Impact factor: 4.132

2.  Instability versus predictability: the molecular diagnosis of myotonic dystrophy.

Authors:  G K Suthers; S M Huson; K E Davies
Journal:  J Med Genet       Date:  1992-11       Impact factor: 6.318

3.  Anticipation legitimized: unstable DNA to the rescue.

Authors:  G R Sutherland; R I Richards
Journal:  Am J Hum Genet       Date:  1992-07       Impact factor: 11.025

Review 4.  Cloning of the gene for the fragile X syndrome: implications for the clinical geneticist.

Authors:  J M Connor
Journal:  J Med Genet       Date:  1991-12       Impact factor: 6.318

5.  Fragile-X syndrome: unique genetics of the heritable unstable element.

Authors:  S Yu; J Mulley; D Loesch; G Turner; A Donnelly; A Gedeon; D Hillen; E Kremer; M Lynch; M Pritchard
Journal:  Am J Hum Genet       Date:  1992-05       Impact factor: 11.025

Review 6.  Anticipation in hereditary disease: the history of a biomedical concept.

Authors:  Judith E Friedman
Journal:  Hum Genet       Date:  2011-06-12       Impact factor: 4.132

Review 7.  The overdue promise of short tandem repeat variation for heritability.

Authors:  Maximilian O Press; Keisha D Carlson; Christine Queitsch
Journal:  Trends Genet       Date:  2014-08-30       Impact factor: 11.639

8.  Epilepsy in families: Age at onset is a familial trait, independent of syndrome.

Authors:  Colin A Ellis; Leonid Churilov; Michael P Epstein; Sharon X Xie; Susannah T Bellows; Ruth Ottman; Samuel F Berkovic
Journal:  Ann Neurol       Date:  2019-05-20       Impact factor: 10.422

9.  Allelic instability in mitosis: a unified model for dominant disorders.

Authors:  C J Zheng; B Byers; S H Moolgavkar
Journal:  Proc Natl Acad Sci U S A       Date:  1993-11-01       Impact factor: 11.205

Review 10.  The unstable repeats--three evolving faces of neurological disease.

Authors:  David L Nelson; Harry T Orr; Stephen T Warren
Journal:  Neuron       Date:  2013-03-06       Impact factor: 17.173

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