Novel Complex Unbalanced Dicentric X-Autosome Rearrangement in a Thoroughbred Mare with a Mild Effect on the Phenotype

Complex structural X chromosome abnormalities are rare in humans and animals, and not recurrent. Yet, each case provides a fascinating opportunity to evaluate X chromosome content and functional status in relation to the effect on the phenotype. Here, we report the first equine case of a complex unb...

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Main Authors: Mendoza, M. N., Schalnus, S. A., Thomson, B., Bellone, R. R., Juras, R., Raudsepp, T.
Format: info:eu-repo/semantics/article
Language:Inglés
Published: Karger 2021
Subjects:
Online Access:https://hdl.handle.net/20.500.12955/1410
https://doi.org/10.1159/000511236
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author Mendoza, M. N.
Schalnus, S. A.
Thomson, B.
Bellone, R. R.
Juras, R.
Raudsepp, T.
author_browse Bellone, R. R.
Juras, R.
Mendoza, M. N.
Raudsepp, T.
Schalnus, S. A.
Thomson, B.
author_facet Mendoza, M. N.
Schalnus, S. A.
Thomson, B.
Bellone, R. R.
Juras, R.
Raudsepp, T.
author_sort Mendoza, M. N.
collection Repositorio INIA
description Complex structural X chromosome abnormalities are rare in humans and animals, and not recurrent. Yet, each case provides a fascinating opportunity to evaluate X chromosome content and functional status in relation to the effect on the phenotype. Here, we report the first equine case of a complex unbalanced X-autosome rearrangement in a healthy but short in stature Thoroughbred mare. Studies of about 200 cells by chromosome banding and FISH revealed an abnormal 2n=63,X,der(X;16) karyotype with a large dicentric derivative chromosome (der). The der was comprised of normal Xp material, a palindromic duplication of Xq12q21, and a translocation of chromosome 16 to the inverted Xq12q21 segment by the centromere, whereas the distal Xq22q29 was deleted from the der. Microsatellite genotyping determined a paternal origin of the der. While there was no option to experimentally investigate the status of X chromosome inactivation (XCI), the observed mild phenotype of this case suggested the following scenario to retain an almost normal genetic balance: active normal X, inactivated X-portion of the der, but without XCI spreading into the translocated chromosome 16. Cases like this present unique resources to acquire information about species-specific features of X regulation and the role of X-linked genes in development, health, and disease.
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institution Institucional Nacional de Innovación Agraria
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spelling INIA14102022-11-21T20:18:10Z Novel Complex Unbalanced Dicentric X-Autosome Rearrangement in a Thoroughbred Mare with a Mild Effect on the Phenotype Mendoza, M. N. Schalnus, S. A. Thomson, B. Bellone, R. R. Juras, R. Raudsepp, T. Complex rearrangement Dicentric Horse Unbalanced X-autosome translocation https://purl.org/pe-repo/ocde/ford#4.04.02 Complex structural X chromosome abnormalities are rare in humans and animals, and not recurrent. Yet, each case provides a fascinating opportunity to evaluate X chromosome content and functional status in relation to the effect on the phenotype. Here, we report the first equine case of a complex unbalanced X-autosome rearrangement in a healthy but short in stature Thoroughbred mare. Studies of about 200 cells by chromosome banding and FISH revealed an abnormal 2n=63,X,der(X;16) karyotype with a large dicentric derivative chromosome (der). The der was comprised of normal Xp material, a palindromic duplication of Xq12q21, and a translocation of chromosome 16 to the inverted Xq12q21 segment by the centromere, whereas the distal Xq22q29 was deleted from the der. Microsatellite genotyping determined a paternal origin of the der. While there was no option to experimentally investigate the status of X chromosome inactivation (XCI), the observed mild phenotype of this case suggested the following scenario to retain an almost normal genetic balance: active normal X, inactivated X-portion of the der, but without XCI spreading into the translocated chromosome 16. Cases like this present unique resources to acquire information about species-specific features of X regulation and the role of X-linked genes in development, health, and disease. Abstract. Introduction. Material and Methods. Results. Discussion. Conclusions and Future Approaches. References. 2021-07-19T18:40:13Z 2021-07-19T18:40:13Z 2020-11-05 info:eu-repo/semantics/article Mendoza M, N, Schalnus S, A, Thomson B, Bellone R, R, Juras R, Raudsepp T. Novel Complex Unbalanced Dicentric X-Autosome Rearrangement in a Thoroughbred Mare with a Mild Effect on the Phenotype. Cytogenet Genome Res 2020;160:597-609. doi: 10.1159/000511236 https://hdl.handle.net/20.500.12955/1410 Cytogenet and Genome Research https://doi.org/10.1159/000511236 eng Cytogenet and Genome Research 2020; 160: 597–609 https://doi.org/10.1159/000511236 info:eu-repo/semantics/openAccess https://creativecommons.org/licenses/by-nc-nd/4.0/ application/pdf application/pdf Estados Unidos Karger Suiza Instituto Nacional de Innovación Agraria Repositorio Institucional - INIA
spellingShingle Complex rearrangement
Dicentric
Horse
Unbalanced
X-autosome translocation
https://purl.org/pe-repo/ocde/ford#4.04.02
Mendoza, M. N.
Schalnus, S. A.
Thomson, B.
Bellone, R. R.
Juras, R.
Raudsepp, T.
Novel Complex Unbalanced Dicentric X-Autosome Rearrangement in a Thoroughbred Mare with a Mild Effect on the Phenotype
title Novel Complex Unbalanced Dicentric X-Autosome Rearrangement in a Thoroughbred Mare with a Mild Effect on the Phenotype
title_full Novel Complex Unbalanced Dicentric X-Autosome Rearrangement in a Thoroughbred Mare with a Mild Effect on the Phenotype
title_fullStr Novel Complex Unbalanced Dicentric X-Autosome Rearrangement in a Thoroughbred Mare with a Mild Effect on the Phenotype
title_full_unstemmed Novel Complex Unbalanced Dicentric X-Autosome Rearrangement in a Thoroughbred Mare with a Mild Effect on the Phenotype
title_short Novel Complex Unbalanced Dicentric X-Autosome Rearrangement in a Thoroughbred Mare with a Mild Effect on the Phenotype
title_sort novel complex unbalanced dicentric x autosome rearrangement in a thoroughbred mare with a mild effect on the phenotype
topic Complex rearrangement
Dicentric
Horse
Unbalanced
X-autosome translocation
https://purl.org/pe-repo/ocde/ford#4.04.02
url https://hdl.handle.net/20.500.12955/1410
https://doi.org/10.1159/000511236
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