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Pathology

Houston, Texas

Cooper Lab
Pathology
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Human CELF/BRUNOL Genes

Cooper1,6
Good2
Notes
Timchenko/
Swanson3
% identity
Sakaki5
CUG-BP
Expression
Chrom.
CUG-BP BRUNOL2 CUG-BP1# --- ubiquitous 11p11 alt spliced, 2 promoters
ETR-3 BRUNOL3 CUG-BP2# NAPOR1,2,3* 78% brain, emb musc 10p13 alt spliced, 5 promoters
CELF3 BRUNOL1   44% brain only 1q21 alt spliced
CELF4 BRUNOL4   42% brain enriched 18q12 alt spliced
CELF5 BRUNOL5   39% brain only 19p13 alt spliced
CELF6 BRUNOL6   46% brain, kidney 15q24 alt spliced

# In early papers, different phosphoisoforms of CUG-BP were called CUG-BP1 (hyperphosphorylated) and CUG-BP2 (hypophosphorylated)4. CUG-BP2 now refers ETR-3/BRUNOL3 in GenBank.

* NAPOR1, NAPOR2, and NAPOR35 have different N-termini due to use of three alternative promoters (see below).

The ETR-3/BRUNOL3/NAPOR gene contains several alternative promoters (first exons) which generate different N-termini

Diagram of CELF

References

1. Ladd, A. N., Charlet-B.N., and T. A. Cooper. 2001. The CELF family of RNA binding proteins is implicated in cell-specific and developmentally regulated alternative splicing. Mol Cell Biol 21:1285-1296.

2. Good, P. J., Q. Chen, S. J. Warner, and D. C. Herring. 2000. A family of human RNA-binding proteins related to the Drosophila Bruno translational regulator. J Biol Chem 275:28583-28592.

3. Timchenko, L. T., J. W. Miller, N. A. Timchenko, D. R. Devore, K. V. Datar, L. J. Lin, R. Roberts, C. T. Caskey, and M. S. Swanson. 1996. Identification of a (CUG)n triplet repeat RNA-binding protein and its expression in myotonic dystrophy. Nucl Acids Res 24:4407-4414.

4. Roberts, R., N.A. Timchenko, J.W. Miller, S. Reddy, C.T. Caskey, M.S. Swanson, and L.T. Timchenko. 1997. Altered phosphorylation and intracellular distribution of a (CUG)n triplet repeat RNA-binding protein in patients with myotonic dystrophy and in myotonin protein kinase knockout mice. Proc. Natl. Acad. Sci. 94:13221-13226.

5. Choi, D. K., T. Ito, F. Tsukahara, M. Hirai, and Y. Sakaki. 1999. Developmentally regulated expression of mNAPOR encoding an apoptosis-induced ELAV-type RNA binding protein. Gene 237:135-142.

6. Ladd, A.N., Nguyen, N.H. Malhotra, K and Cooper, T.A. (2004) CELF6, a member of the CELF family of RNA binding proteins, regulates MSE-dependent alternative splicing. J. Biol. Chem. 279,17756-17764.