Western Blot (WB)
(PABPN1 Antibody (C-term) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the PABPN1 antibody detected the PABPN1 protein (arrow).)
Rabbit PABPN1 Polyclonal Antibody | anti-PABPN1 antibody
PABPN1 Antibody (C-term)
Western Blot (WB)
(PABPN1 Antibody (C-term) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the PABPN1 antibody detected the PABPN1 protein (arrow).)
Western Blot (WB)
(PABPN1 Antibody (C-term) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the PABPN1 antibody detected the PABPN1 protein (arrow).)
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.
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)
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
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
Research Articles on PABPN1
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Product Notes
The PABPN1 pabpn1 (Catalog #AAA9211915) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 270-298. The PABPN1 Antibody (C-term) reacts with Human (Predicted Reactivity: Bovine) and may cross-react with other species as described in the data sheet. AAA Biotech's PABPN1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA). WB~~1:1000. Researchers should empirically determine the suitability of the PABPN1 pabpn1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "PABPN1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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