skip to content »

Pathology

Houston, Texas

Cooper Lab
Pathology
not shown on screen

CLEF Proteins

Return to Cooper Laboratory

Six Human CELF/BRUNOL Genes
Nomenclature          

 

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

# 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.

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

 

Tissue distribution of human CELF family members

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

5' UTR---
-5' UTR--MNGALDHSDQPDP--- S* NAPOR-1
MTVEGRLLVPDRI
NGTANKMNGALDHSDQPDP--- M2* NAPOR-2
MRCPKSAVTMRNEELLLS
NGTANKMNGALDHSDQPDP--- M1* NAPOR-3
MFERTSKPAFVENICVESMRCPKSAVTMRNEELLLS
NGTANKMNGALDHSDQPDP--- L*  
different 5' terminal exons
common exon
intron position

* 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
       
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. 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