Literature DB >> 8929953

A cytogenetic deletion, del(17)(q11.22q21.1), in a patient with sporadic neurofibromatosis type 1 (NF1) associated with dysmorphism and developmental delay.

M Upadhyaya1, S H Roberts, J Maynard, E Sorour, P W Thompson, M Vaughan, A O Wilkie, H E Hughes.   

Abstract

We report the first visible cytogenetic deletion involving the NF1 gene in a patient with sporadic neurofibromatosis, dysmorphic features, and marked developmental delay. The combined evidence of molecular and cytogenetic techniques based on dosage reduction, hemizygosity for microsatellite markers, high resolution G banding, and FISH analysis, predicts this deletion to be approximately 7 Mb in size. Our findings highlight the importance of conducting a detailed cytogenetic and FISH analysis in patients with NF1 who have additional dysmorphic features or particularly severe learning difficulties.

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Year:  1996        PMID: 8929953      PMCID: PMC1051842          DOI: 10.1136/jmg.33.2.148

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


  31 in total

1.  A yeast artificial chromosome contig encompassing the type 1 neurofibromatosis gene.

Authors:  D A Marchuk; R Tavakkol; M R Wallace; B H Brownstein; P Taillon-Miller; C T Fong; E Legius; L B Andersen; T W Glover; F S Collins
Journal:  Genomics       Date:  1992-07       Impact factor: 5.736

2.  Parameters of the human genome.

Authors:  N E Morton
Journal:  Proc Natl Acad Sci U S A       Date:  1991-09-01       Impact factor: 11.205

3.  A major segment of the neurofibromatosis type 1 gene: cDNA sequence, genomic structure, and point mutations.

Authors:  R M Cawthon; R Weiss; G F Xu; D Viskochil; M Culver; J Stevens; M Robertson; D Dunn; R Gesteland; P O'Connell
Journal:  Cell       Date:  1990-07-13       Impact factor: 41.582

4.  Deletions and a translocation interrupt a cloned gene at the neurofibromatosis type 1 locus.

Authors:  D Viskochil; A M Buchberg; G Xu; R M Cawthon; J Stevens; R K Wolff; M Culver; J C Carey; N G Copeland; N A Jenkins
Journal:  Cell       Date:  1990-07-13       Impact factor: 41.582

5.  A 90 kb DNA deletion associated with neurofibromatosis type 1.

Authors:  M Upadhyaya; A Cheryson; W Broadhead; A Fryer; D J Shaw; S Huson; M R Wallace; L B Andersen; D A Marchuk; D Viskochil
Journal:  J Med Genet       Date:  1990-12       Impact factor: 6.318

6.  Large de novo DNA deletion in a patient with sporadic neurofibromatosis 1, mental retardation, and dysmorphism.

Authors:  L M Kayes; V M Riccardi; W Burke; R L Bennett; K Stephens
Journal:  J Med Genet       Date:  1992-10       Impact factor: 6.318

7.  Male-driven evolution of DNA sequences.

Authors:  L C Shimmin; B H Chang; W H Li
Journal:  Nature       Date:  1993-04-22       Impact factor: 49.962

8.  Preferential mutation of the neurofibromatosis type 1 gene in paternally derived chromosomes.

Authors:  K Stephens; L Kayes; V M Riccardi; M Rising; V P Sybert; R A Pagon
Journal:  Hum Genet       Date:  1992-01       Impact factor: 4.132

9.  An Alu polymorphism intragenic to the neurofibromatosis type 1 gene (NF1).

Authors:  G F Xu; L Nelson; P O'Connell; R White
Journal:  Nucleic Acids Res       Date:  1991-07-11       Impact factor: 16.971

10.  Analysis of mutations at the neurofibromatosis 1 (NF1) locus.

Authors:  M Upadhyaya; M Shen; A Cherryson; J Farnham; J Maynard; S M Huson; P S Harper
Journal:  Hum Mol Genet       Date:  1992-12       Impact factor: 6.150

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  9 in total

1.  Mice lacking Nf1 in osteochondroprogenitor cells display skeletal dysplasia similar to patients with neurofibromatosis type I.

Authors:  Weixi Wang; Jeffry S Nyman; Koichiro Ono; David A Stevenson; Xiangli Yang; Florent Elefteriou
Journal:  Hum Mol Genet       Date:  2011-07-14       Impact factor: 6.150

2.  Extensively high load of internal tumors determined by whole body MRI scanning in a patient with neurofibromatosis type 1 and a non-LCR-mediated 2-Mb deletion in 17q11.2.

Authors:  Hildegard Kehrer-Sawatzki; Lan Kluwe; Carsten Fünsterer; Victor-Felix Mautner
Journal:  Hum Genet       Date:  2005-03-18       Impact factor: 4.132

3.  Identification of novel deletion breakpoints bordered by segmental duplications in the NF1 locus using high resolution array-CGH.

Authors:  K K Mantripragada; A-C Thuresson; A Piotrowski; T Díaz de Ståhl; U Menzel; G Grigelionis; R E Ferner; S Griffiths; L Bolund; V Mautner; M Nordling; E Legius; D Vetrie; N Dahl; L Messiaen; M Upadhyaya; C E G Bruder; J P Dumanski
Journal:  J Med Genet       Date:  2005-06-08       Impact factor: 6.318

4.  A clinical variant of neurofibromatosis type 1: familial spinal neurofibromatosis with a frameshift mutation in the NF1 gene.

Authors:  E Ars; H Kruyer; A Gaona; P Casquero; J Rosell; V Volpini; E Serra; C Lázaro; X Estivill
Journal:  Am J Hum Genet       Date:  1998-04       Impact factor: 11.025

5.  Coexistence of multiple rare spinal abnormalities in type 1 neurofibromatosis: a case report and literature review.

Authors:  Chun-Ming Zhao; Wen-Jie Zhang; Ai-Bing Huang; Qian Chen; Yuan-Long He; Wei Zhang; Hui-Lin Yang
Journal:  Int J Clin Exp Med       Date:  2015-10-15

Review 6.  Connective tissue dysplasia in five new patients with NF1 microdeletions: further expansion of phenotype and review of the literature.

Authors:  K A Mensink; R P Ketterling; H C Flynn; R A Knudson; N M Lindor; B A Heese; R J Spinner; D Babovic-Vuksanovic
Journal:  J Med Genet       Date:  2006-02       Impact factor: 6.318

7.  NF1 microdeletion syndrome: refined FISH characterization of sporadic and familial deletions with locus-specific probes.

Authors:  P Riva; L Corrado; F Natacci; P Castorina; B L Wu; G H Schneider; M Clementi; R Tenconi; B R Korf; L Larizza
Journal:  Am J Hum Genet       Date:  2000-01       Impact factor: 11.025

Review 8.  Emerging genotype-phenotype relationships in patients with large NF1 deletions.

Authors:  Hildegard Kehrer-Sawatzki; Victor-Felix Mautner; David N Cooper
Journal:  Hum Genet       Date:  2017-02-17       Impact factor: 4.132

Review 9.  Atypical NF1 Microdeletions: Challenges and Opportunities for Genotype/Phenotype Correlations in Patients with Large NF1 Deletions.

Authors:  Hildegard Kehrer-Sawatzki; Ute Wahlländer; David N Cooper; Victor-Felix Mautner
Journal:  Genes (Basel)       Date:  2021-10-19       Impact factor: 4.096

  9 in total

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