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Volume 271,
Number 8,
Issue of February 23, 1996 pp. 4327-4334
©1996 by The American Society for Biochemistry and Molecular Biology, Inc.
Purification
of Nuclear Proteins from Human HeLa Cells That Bind Specifically to the
Unstable Tandem Repeat (CGG) in the Human FMR1
Gene
(Received for publication, September 28, 1995)
Heidrun
Deissler ,
Annett
Behn-Krappa ,
Walter
Doerfler
Autonomous expansions of trinucleotide repeats with the general
structure 5`-d(CNG) -3` are associated with several
human genetic diseases. We have characterized nuclear proteins binding
to the unstable 5`-d(CGG) -3` repeat. Its expansion
in the human FMR1 gene leads to the fragile X syndrome, one of the most
frequent causes of mental retardation in human males. Electrophoretic
mobility shift assays using nuclear extracts from several human and
other mammalian cell lines and from primary human cells demonstrated
specific binding to double-stranded DNA fragments containing only a
5`-d(CGG) -3` repeat or the repeat and flanking genomic
sequences of the human FMR1 gene. Protein binding was inhibited by
complete methylation of the trinucleotide repeat. The complex formed
with crude nuclear extract apparently did not contain the human
transcription factor Sp1 that binds to a characteristic GC-rich
sequence. A 20-kDa protein involved in specific binding to the
double-stranded 5`-d(CGG) -3` repeat was purified from HeLa
nuclear extracts by DNA affinity chromatography.

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Copyright © 1996 by the American Society for Biochemistry and Molecular Biology.
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