Product Details

Uniprot No.

Target Names

PABPN1

Alternative Names

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

Species Reactivity

Human

Immunogen

A synthesized peptide derived from human PABPN1

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

Clonality

Monoclonal

Isotype

Rabbit IgG

Clone No.

6C3

Purification Method

Affinity-chromatography

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.

Form

Liquid

Tested Applications

ELISA, WB, IHC, IF, FC

Recommended Dilution

Application	Recommended Dilution
WB	1:500-1:5000
IHC	1:50-1:200
IF	1:20-1:200
FC	1:20-1:200

Protocols

ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol
Immunofluorescence (IF) Protocol
Flow Cytometry (FC) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Description

PABPN1, also known as polyadenylate-binding nuclear protein 1, plays an essential role in mRNA processing by stimulating poly(A) polymerase activity and controlling the length of poly(A) tails on newly synthesized mRNA transcripts. This nuclear protein is particularly significant in research exploring epigenetics and nuclear signaling pathways, with mutations in PABPN1 linked to oculopharyngeal muscular dystrophy, making it a valuable target for both basic research and disease-focused investigations.

This recombinant monoclonal antibody, clone 6C3, offers the consistency and reproducibility that demanding experimental workflows require. Generated against a synthetic peptide derived from human PABPN1 and produced using recombinant technology in rabbit host, this antibody provides sequence-defined specificity with minimal lot-to-lot variation, ensuring your results remain comparable across extended studies.

Validation across multiple applications demonstrates this antibody's versatility in your research toolkit. Western blot analysis confirms reliable detection in human cell lines including 293, MCF-7, Raji, and HepG2 whole cell lysates, with the observed band at approximately 50 kDa appearing higher than the predicted molecular weight of 32-38 kDa, likely reflecting post-translational modifications such as glycosylation or phosphorylation common to nuclear proteins. Immunohistochemistry staining has been validated in paraffin-embedded human testis tissue and bladder cancer samples, while immunofluorescence studies in HeLa cells reveal the expected nuclear localization pattern. Flow cytometry applications have also been confirmed using HeLa cells.

Whether investigating mRNA processing mechanisms, exploring nuclear signaling dynamics, or examining PABPN1 expression in disease contexts, this antibody delivers the multi-platform flexibility researchers need to advance their understanding of this critical nuclear factor.

Usage

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

Target Background

Function

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. Subunit of the trimeric poly(A) tail exosome targeting (PAXT) complex, a complex that directs a subset of long and polyadenylated poly(A) RNAs for exosomal degradation. The RNA exosome is fundamental for the degradation of RNA in eukaryotic nuclei. Substrate targeting is facilitated by its cofactor MTREX, which links to RNA-binding protein adapters.

Gene References into Functions

The expression of Ala-expanded-PABPN1 causes the formation of nuclear aggregates before the onset of muscle weakness in oculopharyngeal muscular dystrophy. PMID: 27854203
The purpose of this study was to characterize the type of PABPN1 expanded alleles in a large cohort of Oculopharyngeal muscular dystrophy individuals from Mexico. PMID: 27980005
We propose that the extensive binding of CircPABPN1 to HuR prevents HuR binding to PABPN1 mRNA and lowers PABPN1 translation, providing the first example of competition between a circRNA and its cognate mRNA for an RBP that affects translation. PMID: 28080204
Whereas PABPN1 strongly increases the activity of its cognate poly(A) polymerase in vitro, Pab2 was unable to stimulate Pla1 to any significant extent. PMID: 28096519
The described is the mRNA degradation poly(A) tail exosome targeting (PAXT) connection, which comprises the ZFC3H1 Zn-knuckle protein as a central link between MTR4 and the nuclear poly(A)-binding protein PABPN1. PMID: 27871484
PABPN1 aggregates are able to trap TNNT3 pre-mRNA, driving it outside nuclear speckles, leading to an altered SC35-mediated splicing. PMID: 27507886
The levels of these 6 cytokines were not altered in expPABPN1 carriers at a pre-symptomatic stage, suggesting that this group of cytokines is a potential biomarker for muscle weakness in OPMD. Correlation pattern of expression levels could be a novel measurer for disease state PMID: 27506982
Large cohort study demonstrated that in heterozygous and homozygous patients with oculopharyngeal muscular dystrophy, the mean age at diagnosis and the severity of the clinical symptoms correlate to the number of PABPN1 (GCN) repeats. Homozygous patients showed the worse phenotype, suggesting a gene-dose effect in addition to the repeat number expansion. PMID: 28011929
we found a polyadenylation-dependent 3' end maturation pathway for the human telomerase RNA that relies on the nuclear poly(A)-binding protein PABPN1 and the poly(A)-specific RNase PARN. PMID: 26628368
Many ribozymes were assayed and validated, including four ribozymes targeting the transcript of a disease-causing gene (a mutant version of PABPN1). PMID: 26527730
Studied if the stability of the RNP domain of PABPN1 or domain swapping within the RNP domain may add to fibril formation. PMID: 26267866
This function is mediated by the concerted actions of the nuclear poly(A) binding protein PABPN1, poly(A) polymerase (PAP), and the nuclear exosome complex, a pathway we have named PABPN1 and PAP-mediated RNA decay (PPD) PMID: 26484760
These findings demonstrate a role for PABPN1 in rescuing several cytopathological features of TDP-43 proteinopathy by increasing the turnover of pathologic proteins. PMID: 26130692
PABPN1 inhibits expression of transcripts with pAs near the transcription start site (TSS), a property possibly related to its role in RNA degradation PMID: 25906188
this study provided a systematic phosphorylation analysis of the Fanconi anemia protein PALB2, and have revealed important roles of PALB2 Ser-157 and Ser-376 in driving cellular responses to genotoxic stress. PMID: 26420486
the first step of the cleavage and polyadenylation reaction, mRNA cleavage, is affected in muscles expressing alanine-expanded PABPN1. We propose that impaired cleavage is an early defect in Oculopharyngeal muscular dystrophy. PMID: 25816335
Data indicate that intron 6 of the poly(A)-binding protein nuclear 1 (PABPN1) gene is required for autoregulation. PMID: 25963658
The ability of PABPN1 to promote splicing requires its RNA binding and, to a lesser extent, poly(A)polymerase (PAP) - stimulatory functions. PMID: 25896913
We show that ARIH2 E3-ligase regulates PABPN1 protein accumulation and aggregation PMID: 24486325
Loss of PABPN1, a suppressor of APA, might promote tumor aggressiveness by releasing the cancer cells from microRNA-mediated gene regulation. PMID: 24975429
These results suggest that PABPN1 levels regulate muscle cell aging and oculopharyngeal muscular dystrophy represents an accelerated muscle aging disorder. PMID: 23793615
although function of PABPN1 may be compensated by nuclear translocation of PABP4 and perhaps by increase the cytoplasmic abundance of PABP5, these were not sufficient to prevent apoptosis of cells. PABPN1 may have an anti apoptotic role in mammalian cells PMID: 23300856
Concerning the frequency of the expanded (GCN)11 polymorphism in PABPN1, all 34 patients have the normal allele, correlating genotypic and phenotypic features. PMID: 22231868
we show that PABPN1 promotes lncRNA turnover via a polyadenylation-dependent mechanism PMID: 23166521
Time-lapse experiments in cultured myoblasts confirm nuclear speckles as biogenesis sites of PABPN1 inclusions in oculopharyngeal muscular dystrophy. PMID: 22249111
PABPN1 with a 17 alanine expansion generates stress that initiates apoptosis through a p53-dependent mechanism. PMID: 22519734
Taken together, the present data suggest that heterozygote OPMD patients may show some cognitive impairments and psychological disorders PMID: 21956377
Fibrillar PABPN1 has a structure that differs from native PABPN1 and circumstantial evidence is presented that the C-terminal domain is involved in fibril formation. PMID: 22570486
Results elucidate a novel function for PABPN1 as a suppressor of alternative cleavage and polyadenylation. PMID: 22502866
In myotubes, the ratio of soluble/insoluble expPABPN1 is significantly lower compared with that of the WT protein. PMID: 21854744
Transportin binding might delay methylation of PABPN1 until after nuclear import. PMID: 21808065
A GCG expansion (GCG)11 in polyadenylate-binding protein nuclear 1 gene caused oculopharyngeal muscular dystrophy in a Chinese family. PMID: 21647273
no correlation was found between muscle weakness, the frequency of repeats in the polyadenine binding protein nuclear , and the frequency and size of nuclear inclusions PMID: 21545772
The autosomal dominant form of this disease is caused by short expansions of a (GCG)(6) repeat to (GCG)(8-13) in the PABPN1 gene. PMID: 11689481
In COS-7 cells, predominantly nuclear protein hnRNP A1 and A/B co-localize with mPABPN1 in insoluble intranuclear aggregates. PMID: 12945950
All families carrying the mutation (GCG)(11)(GCA)(3)(GCG) shared a common ancestral haplotype and the age of the mutation was estimated in 37-53 generations by a composite likelihood method. PMID: 15694141
a 64 year old Chinese-Malaysian woman who presented with progressive dysphagia and bilateral ptosis for about 6 years. and showed repeat expansion in one allele to (GCG)9 while normal in the other (GCG)6. PMID: 15725589
Expanded PABPN1, presumably via the toxic effects of its polyalanine tract, leads to inclusion formation and neurodegeneration in both the transgenic mouse and the human. PMID: 15755680
Cytoplasmic targeting of mutant PABPN1 suppresses protein aggregation and toxicity in oculopharyngeal muscular dystrophy. PMID: 16101680
Recruitment of HSP70 and HSC70 into the cell nucleus reduced mutant PABPN1 aggregation in a HeLa cell culture model. PMID: 16239242
PABPN1 has a role in myogenesis PMID: 16378590
Overexpression of either the wild type or mutant PABPN1 slowed down cell proliferation. The slowing down of proliferation together with the occasional occurrence of apoptosis could contribute in vivo to the late onset of this disease. PMID: 16860991
Mutation of the PABPN1 in oculopharyngeal muscular dystrophy (OPMD) provokes premature senescence in dividing myoblasts, that may be due to intranuclear toxic aggregates. PMID: 17005403
the N-terminal domain of PABPN1 has a role in oculopharyngeal muscular dystrophy PMID: 17229142
This is the first report directly indicating that nuclear aggregation in OPMD may reflect an active process by which cells sequester and inactivate the soluble toxic form of expPABPN1. PMID: 17418585
An Italy case of oculopharyngeal muscular dystrophy in PABNPN1 mutation. PMID: 18175083
Wild-type PABPN1 over-expression can reduce mutant PABPN1 toxicity in both cell and mouse models of Oculopharyngeal muscular dystrophy. PMID: 18178579
We report a unique PABPN1 gene mutation in a large Bulgarian family with OPMD. PMID: 18274805
Expression of additional HSPs including HSP27, HSP40, and HSP105 were induced in mutant PABPN1 expressing cells following exposure to the chemicals mentioned above. PMID: 18343218
PABPN1 transgenic nematodes show muscle cell degeneration and abnormal motility. PMID: 18397876

Hide All

Involvement in disease

Oculopharyngeal muscular dystrophy (OPMD)

Subcellular Location

Nucleus. Cytoplasm. Nucleus speckle.

Tissue Specificity

Ubiquitous.

Database Links

HGNC: 8565

OMIM: 164300

KEGG: hsa:8106

STRING: 9606.ENSP00000216727

UniGene: Hs.707712

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Code	CSB-RA569290A0HU
Size	US$210
	Order now
Image	Western Blot Positive WB detected in: 293 whole cell lysate, MCF-7 whole cell lysate, Raji whole cell lysate, HepG2 whole cell lysate All lanes: PABPN1 antibody at 1:2000 Secondary Goat polyclonal to rabbit IgG at 1/50000 dilution Predicted band size: 33, 32, 38 kDa Observed band size: 50 kDa IHC image of CSB-RA569290A0HU diluted at 1:100 and staining in paraffin-embedded human testis tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4℃ overnight. The primary is detected by a Goat anti-rabbit IgG polymer labeled by HRP and visualized using 0.05% DAB. IHC image of CSB-RA569290A0HU diluted at 1:100 and staining in paraffin-embedded human bladder cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4℃ overnight. The primary is detected by a Goat anti-rabbit IgG polymer labeled by HRP and visualized using 0.05% DAB. Immunofluorescence staining of Hela Cells with CSB-RA569290A0HU at 1:50, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeated by 0.2% TritonX-100, and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4℃. Nuclear DNA was labeled in blue with DAPI. The secondary antibody was FITC-conjugated AffiniPure Goat Anti-Rabbit IgG (H+L). Overlay histogram showing Hela cells stained with CSB-RA569290A0HU (red line) at 1:50. The cells were fixed with 70% Ethylalcohol (18h) and then incubated in 10% normal goat serum to block non-specific protein-protein interactions followedby the antibody (1µg/110⁶ cells) for 1 h at 4℃.The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 30min at 4℃. Control antibody (green line) was Rabbit IgG (1µg/110⁶ cells) used under the same conditions. Acquisition of >10,000 events was performed.
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PABPN1 Recombinant Monoclonal Antibody

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