TY - JOUR
T1 - Identification of (CAG)(n) and (CGG)(n) repeat-binding proteins, CAGERs expressed in mature neurons of the mouse brain
AU - Yano, Hiroko
AU - Wang, Bu Er
AU - Ahmad, Ishtiyaque
AU - Zhang, Jianzhong
AU - Abo, Tatsuhiko
AU - Nakayama, Jun
AU - Krempen, Kimberly
AU - Kohwi, Yoshinori
N1 - Funding Information:
We thank our colleagues for critical reading and comments of this manuscript. We thank Ms S. Au, G. Mosley, and Mr. D.S. Hawkes for editing and Drs. Yamasaki and Muroo (Kyouwa Hakkou Co. Ltd., Machida, Japan) for additional amino acid sequence analyses. This work was initiated at the Burnham Institute (formally La Jolla Cancer Research Foundation) and supported by grants from the National Institutes of Health (RO1CA51377) and by funding from the Hereditary Disease Foundation to Y.K.
Funding Information:
The submitted manuscript has been authored by a contractor of the U.S. Government under contract No. DE-AC03-76SF00098. The U.S. Government’s right to retain a nonexclusive royalty-free license in and to the copyright covering this paper, for governmental purposes, is acknowledged. 1The first two authors contributed equally to this paper. 2Present address: NYU Medical Center, NY. 3 Present address: Kegongwei General Hospital, People’s Republic of China. 4Present address: Nagoya University, Japan. 5Present address: Shinshu University, Japan. 6Present address: San Diego State University, CA. 7To whom reprint requests should be addressed at Lawrence Berkeley Laboratory, One Cyclotron Rd., MS 70A-1118, Berkeley, CA 94720. Fax: (510) 486-4545. E-mail: [email protected].
PY - 1999/9/15
Y1 - 1999/9/15
N2 - 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.
AB - 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.
KW - (CAG) and (CGG) repeats
KW - CAGERs
KW - Neurons
KW - Purα/β
KW - Triplet-repeat diseases
UR - http://www.scopus.com/inward/record.url?scp=0033568031&partnerID=8YFLogxK
U2 - 10.1006/excr.1999.4603
DO - 10.1006/excr.1999.4603
M3 - Article
C2 - 10471324
AN - SCOPUS:0033568031
SN - 0014-4827
VL - 251
SP - 388
EP - 400
JO - Experimental Cell Research
JF - Experimental Cell Research
IS - 2
ER -