Anti -PABPN1, CT (PABPN1, PAB2, PABP2, Polyadenylate-binding protein 2, Nuclear poly(A)-binding protein 1, Poly(A)-binding protein II, Polyadenylate-binding nuclear protein 1)

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

Chromosome: 14; NC_000014.8 (23789397..23795394). Location: 14q11.2

Polyclonal

IgG

Rabbit

Human

Affinity Purified
Purified by Protein A affinity chromatography.

Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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.

Antibodies; Abs to Binding Proteins

ELISA (EL/EIA), Western Blot (WB)

Suitable for use in Western Blot, ELISA
Dilution: ELISA: 1:1,000
Western Blot: 1:100-500

32,749 Da[Similar Products]

poly(A) binding protein, nuclear 1

polyadenylate-binding protein 2; poly(A) binding protein 2; poly(A) binding protein II

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 splicing; RNA-binding

Chromosomal Location of Human Ortholog: 14q11.2

Cellular Component: nucleoplasm; cytoplasm; ribonucleoprotein complex; nucleus

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; nuclear mRNA splicing, via spliceosome; RNA processing; 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

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