Literature DB >> 10978355

A new X linked mental retardation (XLMR) syndrome with short stature, small testes, muscle wasting, and tremor localises to Xq24-q25.

D A Cabezas1, R Slaugh, F Abidi, J F Arena, R E Stevenson, C E Schwartz, H A Lubs.   

Abstract

METHODS: A large family is described in which mental retardation segregates as an X linked trait. Six affected males in three generations were studied by linkage and clinical examination.
RESULTS: Characteristic clinical features include short stature, prominent lower lip, small testes, muscle wasting of the lower legs, kyphosis, joint hyperextensibility, abnormal gait, tremor, and decreased fine motor coordination. Affected subjects also had impaired speech and decreased attention span. A carrier female was mildly affected. A similar disorder was not found on review of our XLMR Database of 124 syndromes. Linkage analysis of 37 markers resulted in a lod score of 2.80 at DXS1212 and 2.76 at DXS425. The limiting markers were DXS424 and DXS1047. Ten of 124 XLMR syndromes and eight of 58 MRX families overlap this region.
CONCLUSIONS: In summary, this family appears to have a new XLMR syndrome localising to Xq24-q25.

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Year:  2000        PMID: 10978355      PMCID: PMC1734699          DOI: 10.1136/jmg.37.9.663

Source DB:  PubMed          Journal:  J Med Genet        ISSN: 0022-2593            Impact factor:   6.318


  13 in total

1.  Allan-Herndon syndrome. II. Linkage to DNA markers in Xq21.

Authors:  C E Schwartz; J Ulmer; A Brown; I Pancoast; H O Goodman; R E Stevenson
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2.  Automation of genetic linkage analysis using fluorescent microsatellite markers.

Authors:  D C Mansfield; A F Brown; D K Green; A D Carothers; S W Morris; H J Evans; A F Wright
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Review 3.  XLMR genes: update 1996.

Authors:  H A Lubs; P Chiurazzi; J F Arena; C Schwartz; L Tranebjaerg; G Neri
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Review 4.  XLMR genes: update 1998.

Authors:  H Lubs; P Chiurazzi; J Arena; C Schwartz; L Tranebjaerg; G Neri
Journal:  Am J Med Genet       Date:  1999-04-02

5.  A comprehensive genetic map of the human genome based on 5,264 microsatellites.

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Journal:  Nature       Date:  1996-03-14       Impact factor: 49.962

6.  Faster sequential genetic linkage computations.

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8.  Spastic paraplegia with iron deposits in the basal ganglia: a new X-linked mental retardation syndrome.

Authors:  J F Arena; C Schwartz; R Stevenson; L Lawrence; A Carpenter; R Duara; D Ledbetter; T Huang; T Lehner; J Ott
Journal:  Am J Med Genet       Date:  1992 Apr 15-May 1

9.  Familial X-linked mental retardation and isolated growth hormone deficiency: clinical and molecular findings.

Authors:  B C Hamel; A P Smits; B J Otten; B van den Helm; H H Ropers; E C Mariman
Journal:  Am J Med Genet       Date:  1996-07-12

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Authors:  G Turner; A Gedeon; J Mulley; G Sutherland; J Rae; K Power; I Arthur
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