Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
4
pubmed:dateCreated
1994-5-10
pubmed:databankReference
pubmed:abstractText
A t(X;14)(q28;q11) translocation was present for many years in T cells in two patients with ataxia telangiectasia (A-T), who subsequently developed T-prolymphocytic leukemia. We describe here the relationship between the translocation breakpoints in these patients with respect to two recently described genes, c6.1A and c6.1B, on Xq28 which are transcribed in opposite directions from the same CpG island. In our first patient, the Xq28 breakpoint disrupts the c6.1A gene which is consequently transcribed as a fusion mRNA with the TCR C alpha chain gene. In the second case, the Xq28 breakpoint lies within the adjacent gene c6.1B, and c6.1A is not transcribed. We show that the c6.1B gene is transcribed in both of our patients. c6.1B may be important in the initial clonal proliferation of T lymphocytes which commonly precedes transformation to T-PLL in ataxia telangiectasia patients. The same gene may also be involved in the development of T-PLL in the non-A-T population.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Apr
pubmed:issn
0887-6924
pubmed:author
pubmed:issnType
Print
pubmed:volume
8
pubmed:geneSymbol
c6.1A, c6.1B
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
564-73
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
1994
pubmed:articleTitle
A gene on chromosome Xq28 associated with T-cell prolymphocytic leukemia in two patients with ataxia telangiectasia.
pubmed:affiliation
Department of Cancer Studies, University of Birmingham, UK.
pubmed:publicationType
Journal Article, Case Reports, Research Support, Non-U.S. Gov't