Document Detail


A palindrome-mediated recurrent translocation with 3:1 meiotic nondisjunction: the t(8;22)(q24.13;q11.21).
MedLine Citation:
PMID:  20673865     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Palindrome-mediated genomic instability has been associated with chromosomal translocations, including the recurrent t(11;22)(q23;q11). We report a syndrome characterized by extremity anomalies, mild dysmorphia, and intellectual impairment caused by 3:1 meiotic segregation of a previously unrecognized recurrent palindrome-mediated rearrangement, the t(8;22)(q24.13;q11.21). There are at least ten prior reports of this translocation, and nearly identical PATRR8 and PATRR22 breakpoints were validated in several of these published cases. PCR analysis of sperm DNA from healthy males indicates that the t(8;22) arises de novo during gametogenesis in some, but not all, individuals. Furthermore, demonstration that de novo PATRR8-to-PATRR11 translocations occur in sperm suggests that palindrome-mediated translocation is a universal mechanism producing chromosomal rearrangements.
Authors:
Molly B Sheridan; Takema Kato; Chad Haldeman-Englert; G Reza Jalali; Jeff M Milunsky; Ying Zou; Ruediger Klaes; Georgio Gimelli; Stefania Gimelli; Robert M Gemmill; Harry A Drabkin; April M Hacker; Julia Brown; David Tomkins; Tamim H Shaikh; Hiroki Kurahashi; Elaine H Zackai; Beverly S Emanuel
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2010-07-30
Journal Detail:
Title:  American journal of human genetics     Volume:  87     ISSN:  1537-6605     ISO Abbreviation:  Am. J. Hum. Genet.     Publication Date:  2010 Aug 
Date Detail:
Created Date:  2010-08-10     Completed Date:  2010-09-01     Revised Date:  2011-07-25    
Medline Journal Info:
Nlm Unique ID:  0370475     Medline TA:  Am J Hum Genet     Country:  United States    
Other Details:
Languages:  eng     Pagination:  209-18     Citation Subset:  IM    
Affiliation:
The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
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MeSH Terms
Descriptor/Qualifier:
AT Rich Sequence / genetics
Adolescent
Adult
Base Sequence
Child
Child, Preschool
Chromosome Breakage
Chromosomes, Human, Pair 22 / genetics*
Chromosomes, Human, Pair 8 / genetics*
Female
Gene Dosage / genetics
Genotype
Health
Humans
Inverted Repeat Sequences / genetics*
Male
Meiosis / genetics*
Molecular Sequence Data
Nondisjunction, Genetic*
Phenotype
Sequence Analysis, DNA
Spermatogenesis / genetics
Spermatozoa / metabolism
Translocation, Genetic / genetics*
Grant Support
ID/Acronym/Agency:
CA39926/CA/NCI NIH HHS; HD26979/HD/NICHD NIH HHS
Comments/Corrections

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