CLEF Proteins
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Six
Human CELF/BRUNOL Genes
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| Nomenclature | ||||||
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Cooper1 |
Good2 |
Timchenko/ Swanson3 |
Sakaki5 |
% identity to CUG-BP |
Chrom. |
Notes |
| CUG-BP | BRUNOL2 | CUG-BP1# | --- | 11p11 | alt spliced, 2 promoters | |
| ETR-3 | BRUNOL3 | CUG-BP2# | NAPOR1, 2, 3% | 78% | 10p13 | alt spliced, 5 promoters |
| CELF3 | BRUNOL1 | 44% | 1q21 | |||
| CELF4 | BRUNOL4 | 42% | 18q12 | alt spliced | ||
| CELF5 | BRUNOL5 | 39% | 19p13 | |||
| CELF6 | BRUNOL6 | 46% | 15q24 | alt spliced | ||
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# In early papers, different phosphoisoforms of CUG-BP were called CUG-BP1 (hyperphosphorylated) and CUG-BP2 (hypophosphorylated)4. Later, CUG-BP2 was used to refer to ETR-3/BRUNOL3. |
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| % NAPOR1, NAPOR2, and NAPOR35 have different N-termini due to use of three alternative promoters (see below). | ||||||
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Tissue distribution of human CELF family members |
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| CUG-BP | multiple tissues (see western below) | |||||
| ETR-3 | multiple tissues, enriched in skeletal muscle and brain (see western below) | |||||
| CELF3 | brain only | |||||
| CELF4 | multiple tissues, enriched in skeletal muscle (see western below) | |||||
| CELF5 | brain only | |||||
| CELF6 | brain, kidney | |||||
| Western Blot of mouse tissues (from ref. #1) | ||||||

The ETR-3/BRUNOL3/NAPOR gene contains several alternative promoters (first exons) which generate different N-termini
The 5' end of the human ETR-3/BRUNOL3/NAPOR gene (partial)

Different 5' terminal exons result in divergent N-termini
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5'
UTR---
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-5' UTR--MNGALDHSDQPDP--- S* | NAPOR-1 |
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MTVEGRLLVPDRI
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NGTANKMNGALDHSDQPDP--- M2* | NAPOR-2 | |
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MRCPKSAVTMRNEELLLS
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NGTANKMNGALDHSDQPDP--- M1* | NAPOR-3 | |
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MFERTSKPAFVENICVESMRCPKSAVTMRNEELLLS
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NGTANKMNGALDHSDQPDP--- L* | ||
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different
5' terminal exons
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common exon |
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intron position
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* S, M2, M1, and L is our in-house nomenclature for the different N termini
| N-termini of ETR-3/BRUNOL3/NAPOR proteins in the database | |||
| Name (Species) | N-terminus | Name (Species) | N-terminus |
| BRUNOL3 (human)2 | S | mNAPOR1 (mouse)5 | S |
| Etr-R3a (rat)6 | S | mNAPOR3 (mouse)5 | M1 |
| Etr-R3b (rat)7 | L | NAPOR1 (human)8 | S |
| ETR-3 (human)1 | S | NAPOR2 (human)8 | M2 |
| ETR-3 (mouse)3 | S | NAPOR3 (human)8 | M1 |
| Etr-3 (Xenopus) | L | NAPOR3 (rat)9 | M1 |
| NAPOR (zebrafish)10 | S | ||
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. Genbank accession number CAA09102
7. Genbank accession number CAA09103
8. Li, D., L. L. Bachinski, and R. Roberts. 2001. Genomic organization and isoform-specific tissue expression of human NAPOR (CUG-BP2) as a candidate gene for familial arrhythmogenic right ventricular dysplasia. Genomics 74:396-401.
9. Genbank accession number AAD13762
10. Genbank accession number AAK52851