Polyadenylate-binding protein 2; PABP-2; Poly(A)-binding protein 2; Nuclear poly(A)-binding protein 1; Poly(A)-binding protein II; PABII; Polyadenylate-binding nuclear protein 1; PABPN1; PAB2; PABP2

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This PABPN1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 270-298 amino acids from the C-terminal region of human PABPN1.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-PABPN1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

This gene encodes an abundant nuclear protein that binds
with high affinity to nascent poly(A) tails. The protein is
required for progressive and efficient polymerization of poly(A)
tails on the 3' ends of eukaryotic genes and controls the size of
the poly(A) tail to about 250 nt. At steady-state, this protein is
localized in the nucleus whereas a different poly(A) binding
protein is localized in the cytoplasm. An expansion of the
trinucleotide (GCG) repeat from normal 6 to 8-13 at the 5' end of
the coding region of this gene leads to autosomal dominant
oculopharyngeal muscular dystrophy (OPMD) disease. Multiple splice
variants have been described but their full-length nature is not
known. One splice variant includes introns 1 and 6 but no protein
is formed.

Western Blot (WB), ELISA (EIA)

WB~~1:1000

PABPN1 Antibody (C-term) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the PABPN1 antibody detected the PABPN1 protein (arrow).

32749

poly(A) binding protein, nuclear 1

polyadenylate-binding protein 2

Polyadenylate-binding protein 2

PAB2; PABP2; PABP-2; Poly(A)-binding protein 2; PABII??[Similar Products]

PABP2_HUMAN

This gene encodes an abundant nuclear protein that binds with high affinity to nascent poly(A) tails. The protein is required for progressive and efficient polymerization of poly(A) tails at the 3' ends of eukaryotic transcripts and controls the size of the poly(A) tail to about 250 nt. At steady-state, this protein is localized in the nucleus whereas a different poly(A) binding protein is localized in the cytoplasm. This gene contains a GCG trinucleotide repeat at the 5' end of the coding region, and expansion of this repeat from the normal 6 copies to 8-13 copies leads to autosomal dominant oculopharyngeal muscular dystrophy (OPMD) disease. Related pseudogenes have been identified on chromosomes 19 and X. Read-through transcription also exists between this gene and the neighboring upstream BCL2-like 2 (BCL2L2) gene. [provided by RefSeq, Dec 2010]

PABPN1: Involved in the 3'-end formation of mRNA precursors (pre-mRNA) by the addition of a poly(A) tail of 200-250 nt to the upstream cleavage product. Stimulates poly(A) polymerase (PAPOLA) conferring processivity on the poly(A) tail elongation reaction and controls also the poly(A) tail length. Increases the affinity of poly(A) polymerase for RNA. Is also present at various stages of mRNA metabolism including nucleocytoplasmic trafficking and nonsense-mediated decay (NMD) of mRNA. Cooperates with SKIP to synergistically activate E-box-mediated transcription through MYOD1 and may regulate the expression of muscle-specific genes. Binds to poly(A) and to poly(G) with high affinity. May protect the poly(A) tail from degradation. Defects in PABPN1 are the cause of oculopharyngeal muscular dystrophy (OPMD). OPMD is a form of late- onset slowly progressive myopathy characterized by eyelid ptosis, dysphagia and, sometimes by other cranial and limb-muscle involvement. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: RNA-binding; RNA splicing

Chromosomal Location of Human Ortholog: 14q11.2

Cellular Component: nucleoplasm; cytoplasm; nucleus; ribonucleoprotein complex

Molecular Function: protein binding; RNA binding; nucleotide binding

Biological Process: transcription from RNA polymerase II promoter; RNA splicing; modification by virus of host mRNA processing; viral infectious cycle; RNA processing; nuclear mRNA splicing, via spliceosome; poly(A)+ mRNA export from nucleus; modification by virus of host cellular process; muscle contraction; virus-host interaction; gene expression; mRNA 3'-end processing; termination of RNA polymerase II transcription

Disease: Oculopharyngeal Muscular Dystrophy

Hosgood, H.D. III, et al. Occup Environ Med 66(12):848-853(2009)
Hurt, J.A., et al. J. Cell Biol. 185(2):265-277(2009)
Kuo, H.C., et al. J. Neurol. Sci. 278 (1-2), 21-24 (2009) :
Tavanez, J.P., et al. PLoS ONE 4 (7), E6418 (2009) :
Maksimova, N.R., et al. Zh Nevrol Psikhiatr Im S S Korsakova 108(6):52-60(2008)

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.