Identification of (CAG)(n) and (CGG)(n) repeat-binding proteins, CAGERs expressed in mature neurons of the mouse brain

Hiroko Yano, Bu Er Wang, Ishtiyaque Ahmad, Jianzhong Zhang, Tatsuhiko Abo, Jun Nakayama, Kimberly Krempen, Yoshinori Kohwi

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11 Scopus citations


The trinucleotide repeats (CAG)(n) and (CGG)(n) have been shown to be expanded in responsible genes of several human hereditary neurological disorders. In studies of mice, we previously identified two homologous single-stranded (ss)(CAG) and ss(CGG) repeat-binding proteins, CAGER-1 (44 kDa) and CAGER-2 (40 kDa) (CAG-element-recognizing proteins). The specific binding activities of these proteins were predominantly detected in the mouse brain. We have isolated the cDNAs encoding CAGER-1 and CAGER-2 and found that they were identical to previously reported cDNAs for Purα and Purβ, respectively. Purα of 28 kda was previously identified as a replication- origin-binding protein that is ubiquitously expressed in proliferating cells. We show that the transcripts of CAGERs increase after birth and are detected at high levels in the adult mouse brain but at very low or virtually undetectable levels in other mouse tissues. Biochemical properties and molecular weights are different between CAGERS and Purα/β. Immunostaining with specific antibodies against CAGERS indicates that CAGERS in the mouse brain reside in nonproliferating neurons but not in proliferating glia. We conclude that CAGERS and Purα/β are unrelated proteins, and CAGERS are neuronal single-stranded sequence-binding proteins in the mouse brain. Misassignment of cDNAs is described.

Original languageEnglish
Pages (from-to)388-400
Number of pages13
JournalExperimental Cell Research
Issue number2
StatePublished - Sep 15 1999


  • (CAG) and (CGG) repeats
  • CAGERs
  • Neurons
  • Purα/β
  • Triplet-repeat diseases


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