Literature DB >> 24863632

Mutation in the X-linked RAB40AL gene (Martin-Probst syndrome) with mental retardation, sensorineural hearing loss, and anomalies of the craniofacies and genitourinary tract: a second case report.

James Lee1, Stacey Wong, Richard G Boles.   

Abstract

UNLABELLED: An X-linked neurodevelopmental disorder previously had been reported in only one family, associated with a p.D59G mutation in the RAB40AL gene that encodes a mitochondrial Ras protein. The three related males described had varying degrees of cognitive impairment, sensorineural hearing loss, short stature, dysmorphic facies, and other morphological defects.
CONCLUSION: We herein present an unrelated 20-year-old male with similar manifestations also with p.D59G in the RAB40AL gene, which supports the existence of this condition previously coined as Martin-Probst syndrome (OMIM: 300519).

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Year:  2014        PMID: 24863632     DOI: 10.1007/s00431-014-2339-x

Source DB:  PubMed          Journal:  Eur J Pediatr        ISSN: 0340-6199            Impact factor:   3.183


  3 in total

1.  Skewed X-inactivation in carriers establishes linkage in an X-linked deafness-mental retardation syndrome.

Authors:  Frank J Probst; Peter Hedera; Anthony M Sclafani; Maria Grazia Pomponi; Giovanni Neri; Jessica Tyson; Julie A Douglas; Elizabeth M Petty; Donna M Martin
Journal:  Am J Med Genet A       Date:  2004-12-01       Impact factor: 2.802

2.  Characterisation and genetic mapping of a new X linked deafness syndrome.

Authors:  D M Martin; F J Probst; S A Camper; E M Petty
Journal:  J Med Genet       Date:  2000-11       Impact factor: 6.318

3.  Disruption of RAB40AL function leads to Martin--Probst syndrome, a rare X-linked multisystem neurodevelopmental human disorder.

Authors:  Jirair Krikor Bedoyan; Valerie M Schaibley; Weiping Peng; Yongsheng Bai; Kajari Mondal; Amol C Shetty; Mark Durham; Joseph A Micucci; Arti Dhiraaj; Jennifer M Skidmore; Julie B Kaplan; Cindy Skinner; Charles E Schwartz; Anthony Antonellis; Michael E Zwick; James D Cavalcoli; Jun Z Li; Donna M Martin
Journal:  J Med Genet       Date:  2012-05       Impact factor: 6.318

  3 in total
  2 in total

1.  A note of caution on the diagnosis of Martin-Probst syndrome by the detection of the p.D59G mutation in the RAB40AL gene.

Authors:  Monika Ołdak; Ewelina Ruszkowska; Agnieszka Pollak; Agnieszka Sobczyk-Kopcioł; Cezary Kowalewski; Aleksandra Piwońska; Wojciech Drygas; Rafał Płoski
Journal:  Eur J Pediatr       Date:  2014-11-05       Impact factor: 3.183

2.  X-exome sequencing of 405 unresolved families identifies seven novel intellectual disability genes.

Authors:  H Hu; S A Haas; J Chelly; H Van Esch; M Raynaud; A P M de Brouwer; S Weinert; G Froyen; S G M Frints; F Laumonnier; T Zemojtel; M I Love; H Richard; A-K Emde; M Bienek; C Jensen; M Hambrock; U Fischer; C Langnick; M Feldkamp; W Wissink-Lindhout; N Lebrun; L Castelnau; J Rucci; R Montjean; O Dorseuil; P Billuart; T Stuhlmann; M Shaw; M A Corbett; A Gardner; S Willis-Owen; C Tan; K L Friend; S Belet; K E P van Roozendaal; M Jimenez-Pocquet; M-P Moizard; N Ronce; R Sun; S O'Keeffe; R Chenna; A van Bömmel; J Göke; A Hackett; M Field; L Christie; J Boyle; E Haan; J Nelson; G Turner; G Baynam; G Gillessen-Kaesbach; U Müller; D Steinberger; B Budny; M Badura-Stronka; A Latos-Bieleńska; L B Ousager; P Wieacker; G Rodríguez Criado; M-L Bondeson; G Annerén; A Dufke; M Cohen; L Van Maldergem; C Vincent-Delorme; B Echenne; B Simon-Bouy; T Kleefstra; M Willemsen; J-P Fryns; K Devriendt; R Ullmann; M Vingron; K Wrogemann; T F Wienker; A Tzschach; H van Bokhoven; J Gecz; T J Jentsch; W Chen; H-H Ropers; V M Kalscheuer
Journal:  Mol Psychiatry       Date:  2015-02-03       Impact factor: 15.992

  2 in total

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