CUGBP Elav-like family member 2 Rabbit Polyclonal Antibody (R1403-3)

UNIPROT #

O95319

PROTEIN NAME

CUGBP Elav-like family member 2

SYNONYMS

Bruno-like protein 3 antibody;BRUNOL3 antibody;Cardiac elav type RNA binding protein antibody;CELF-2 antibody;celf2 antibody;CELF2_HUMAN antibody;CUG BP2 antibody;CUG triplet repeat RNA-binding protein 2 antibody;CUG-BP- and ETR-3-like factor 2 antibody;CUG-BP2 antibody;CUGBP Elav like family member 2 antibody;CUGBP Elav-like family member 2 antibody;CUGBP2 antibody;dJ323N1.1 antibody;elav type RNA binding protein antibody;ELAV-type RNA-binding protein 3 antibody;ETR 3 antibody;ETR-3 antibody;ETR3 antibody;HGNC:2550 antibody;hNAPOR antibody;NAPOR 2 antibody;NAPOR antibody;Neuroblastoma apoptosis-related RNA-binding protein antibody;Neuroplastoma apoptosis related RNA binding protein 3 antibody;RNA binding protein BRUNOL3 antibody;RNA-binding protein BRUNOL-3 antibody

SEQUENCE SIMILARITIES

Belongs to the CELF/BRUNOL family.

TISSUE SPECIFICITY

Expressed in frontal cortex. Isoform 1 is expressed in brain and lung. Isoform 2 is expressed in heart, brain, placenta, lung, liver, kidney, skeletal muscle and pancreas. Isoform 4 is expressed in heart, lung, skeletal muscle, kidney and pancreas.

DEVELOPMENTAL STAGE

Isoform 1 is expressed in fetal brain. Isoform 2 is expressed in fetal heart, brain, thymus, lung, liver, skeletal muscle, kidney and spleen. Isoform 4 is expressed in fetal heart, brain, thymus, lung and skeletal muscle.

SUBCELLULAR LOCATION

Nucleus. Cytoplasm.

FUNCTION

Myotonic dystrophy (DM) is an autosomal dominant neuromuscular disease that is associated with a (CTG)n repeat expansion in the 3'-untranslated region of the myotonin protein kinase gene (DMPK). CUG-BP1 and CUG-BP2 are proteins that bind specifically to (CUG)8 oligonucleotides in vitro. While CUG-BP1 has the major binding activity in normal cells, nuclear CUG-BP2 binding activity increases in DM cells. Both CUG-BP1 and CUG-BP2 are isoforms of a novel heterogeneous nuclear ribonucleoprotein (hnRNP), hNab50. CUG-BP1, an RNA CUG triplet repeat binding protein, regulates splicing and translation of various RNAs. Expansion of RNA CUG repeats in the DMPK in DM is associated with alterations in binding activity of CUG-BP1 as well as alterations in the translation of the C/EBPb transcription factor. CUG-BP1 is an important regulator of initiation from different AUG codons of C/EBPb mRNA. In normal cells, CUG-BP1 up-regulates the p21 protein during differentiation by inducing the translation of p21 via binding to a GC-rich sequence located within the 5' region of p21 mRNA. In DM cells, failure to accumulate CUG-BP1 leads to a reduction of p21 and alterations in other proteins responsible for cell cycle withdrawl.