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UBQLN2 Antibody – Mouse Monoclonal

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS415251

MBS415251 is an antibody targeting UBQLN2. Also known as UBQLN2 Antibody. Reactive in Human, mouse. Host: Mouse. More details

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Product Documentation
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Sample Manual Insert View PDF
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Testing Data View PDF
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Material Safety Data Sheet View PDF
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Technical Datasheet View PDF
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Product Overview

Product Name
UBQLN2, Monoclonal Antibody
Full Product Name
UBQLN2 Antibody
Product Gene Name
anti-UBQLN2 antibody
Product Synonym Names
UBQLN2
Clonality
Monoclonal
Isotype
IgG1
Host
Mouse
Species Reactivity
Human, mouse
Form/Format
Liquid
Purity/Purification
Affinity purified
Concentration
100ug/100ul (lot specific)
Preparation and Storage
Store at -20 degree C/1 year
Molecular Weight
65,696 Da
Chromosome Location
Chromosome: X; NC_000023.10 (56590025..56593443). Location: Xp11.21
OMIM
300264
3D Structure
ModBase 3D Structure for Q9UHD9
Immunogen
Ubiquilin 2, also known as PLIC2 and Chap1, is a member of the ubiquilin protein family, which regulate the degradation of cellular proteins through proteasome or autophage-like pathways (1, 2, 3). Humans have four ubiquilin genes, each encoding a separate protein referred to as Ubiquilin 1, 2, 3 and 4. All ubiquilins contain an N-terminal ubiquitin-like (UBL) domain and a C-terminal ubiquitin-associated (UBA) domain, while the central part of the molecules are highly variable. The UBL domains bind subunits of the proteasome, and the UBA domains binds to polyubiquitin chains that are typically conjugated onto proteins marked for proteosomal degradation (1). Ubiquilin 2 has a unique region close to the C terminus containing 12 PXX tandem collagen like repeats, where P is proline and X is most cases valine, glycine, isoleucine or threonine. Teepu Siddique and his collaborators have identified mutations in the ubiquilin 2 gene leading to protein point mutations which were important contributors to several forms of amyotrophic lateral sclerosis (ALS) and Frontotemporal lobar degeneration (FTLD). Interestingly, these mutations involved alterations in proline residues in the PXX repeat region (P497H, P497S, P506T, P509S and P525S, ref. 4). Recently, the Lee and Trojanowski group investigated C9orf72 hexanucleotide expansion and ubiquilin 2 pathology in patients with ALS and FTLD by genetic analysis and immunohistochemistry and found distinct ubiquilin 2 pathology in ALS and FTLD-TDP with C9orf72 expansion (5). C9orf72 hexonucleotide expansion is the most commmon cause to date of familial ALS and FTLD (6, 7). Ubiquilin 2 protein is of different molecular size in mouse and human, 638 and 624 amino acids respectively. As a result the mouse protein, endogenously expressed in rodent 3T3 cells, runs on SDS-PAGE and western blots slightly slower than the human protein. Our antibody MCA-6H9 was raised against human ubiquilin 2 expressed in and purified from E. coli. The HGNC name for this protein is UBQLN2.
Storage Buffer
PBS, pH 7.4 with 0.02% sodium azide.
Applications Tested/Suitable for anti-UBQLN2 antibody
Immunofluorescence (IF), Western Blot (WB)
Application Notes for anti-UBQLN2 antibody
Try at dilutions of ~1:1,000 for immunofluorescence. For western blots try at 1:1,000-2,000.

Western Blot (WB) of anti-UBQLN2 antibody

Mouse Anti-UBQLN2 Monoclonal Antibody – Cat# MBS415251

Product References and Citations for anti-UBQLN2 antibody

1. Kleijnen MF, Shih AH, Zhou P, Kumar S, Soccio R E, Kedersha N L, Gill G, Howley PM. The hPLIC proteins may provide a link between the ubiquitination machinery and the proteasome. Molec. Cell 6: 409-419 (2000).2. N'Diaye EN, Kajihara KK, Hsieh I, Morisaki H, Debnath J, Brown EJ. PLIC proteins or ubiquilins regulate autophagy-dependent cell survival during nutrient starvation. EMBO Rep. 10:173-9 (2009).3. Rothenberg C, Srinivasan D, Mah L, Kaushik S, Peterhoff CM, Ugolino J, Fang S, Cuervo AM, Nixon RA, Monteiro MJ. Ubiquilin functions in autophagy and is degraded by chaperone-mediated autophagy. Hum Mol Genet. Aug 15;19 (16): 3219-32. Epub Jun 7 (2010).4. Deng HX, Chen W, Hong ST, Boycott KM, Gorrie GH, Siddique N, Yang Y, Fecto F, Shi Y, Zhai H, Jiang H, Hirano M, Rampersaud E, Jansen GH, Donkervoort S, Bigio EH, Brooks BR, AjroudK, Sufit RL, Haines JL, Mugnaini E, Pericak-Vance MA, Siddique T. Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia. Nature Aug 21;477(7363):211-5 （2011).5. Brettschneider J, Van Deerlin VM, Robinson JL, Kwong L, Lee EB, Ali YO, Safren N, Monteiro MJ, Toledo JB, Elman L, McCluskey L, Irwin DJ, Grossman M, Molina-Porcel L, Lee VM, Trojanowski JQ. Pattern of ubiquilin pathology in ALS and FTLD indicates presence of c9orf72 hexanucleotide expansion. Acta Neuropathol. Jun;123 (6):825-39 （2012).6. Renton AE, Majounie E, Waite AA, et al. Hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron. Oct 20;72(2):257-68 (2011).7. DeJesus-Hernandez M, Mackenzie IR, Boeve BF, Boxer AL, Baker M, Rutherford NJ, Nicholson AM, Finch NA, Flynn H, Adamson J, Kouri N, Wojtas A, Sengdy P, Hsiung GY, Karydas A, Seeley WW, Josephs KA, Coppola G, Geschwind DH, Wszolek ZK, Feldman H, Knopman DS, Petersen RC, Miller BL, Dickson DW, Boylan KB, Graff-Radford NR, Rademakers R. Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron.Oct 20;72(2):245-56 (2011).

Other Notes

Small volumes of anti-UBQLN2 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.

Additional Details

NCBI/Uniprot data below describe general gene information for UBQLN2. It may not necessarily be applicable to this product.

NCBI GI #
16753207
NCBI GeneID
29978
NCBI Accession #
NP_038472.2
UniProt Primary Accession #
Q9UHD9
UniProt Secondary Accession #
O94798; Q5D027; Q9H3W6; Q9HAZ4
UniProt Related Accession #
Q9UHD9
GenBank Nucleotide #
NM_013444.3
NCBI Official Full Name
ubiquilin-2
NCBI Official Synonym Full Names
ubiquilin 2
NCBI Official Symbol
UBQLN2
NCBI Official Synonym Symbols
DSK2; ALS15; CHAP1; N4BP4; PLIC2; HRIHFB2157
UniProt Protein Name
Ubiquilin-2
UniProt Synonym Protein Names
Chap1; DSK2 homolog; Protein linking IAP with cytoskeleton 2; PLIC-2; hPLIC-2; Ubiquitin-like product Chap1/Dsk2
Protein Family
Ubiquilin
UniProt Gene Name
UBQLN2
UniProt Synonym Gene Names
N4BP4; PLIC2; PLIC-2; hPLIC-2
UniProt Entry Name
UBQL2_HUMAN
NCBI Protein Information
ubiquilin-2; Nedd4 binding protein 4; ubiquitin-like product Chap1/Dsk2; protein linking IAP with cytoskeleton 2

NCBI Summary for UBQLN2

This gene encodes an ubiquitin-like protein (ubiquilin) that shares high degree of similarity with related products in yeast, rat and frog. Ubiquilins contain a N-terminal ubiquitin-like domain and a C-terminal ubiquitin-associated domain. They physically associate with both proteasomes and ubiquitin ligases; and thus, are thought to functionally link the ubiquitination machinery to the proteasome to affect in vivo protein degradation. This ubiquilin has also been shown to bind the ATPase domain of the Hsp70-like Stch protein. [provided by RefSeq, Oct 2009]
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UniProt Comments for UBQLN2

Function: Increases the half-life of proteins destined to be degraded by the proteasome; may modulate proteasome-mediated protein degradation. Ref.2

Subunit structure: Binds UBE3A and BTRC. Interacts with the 19S proteasome subunit. Ref.1 Ref.2

Subcellular location: Cytoplasm. Nucleus. Note: Where it colocalizes with the proteasome. Associated with fibers in mitotic cells. Ref.2 Ref.7

Induction: Highly expressed in mitotic cells from metaphase to telophase. Expression in non-mitotic cells is very low.

Involvement in disease: Amyotrophic lateral sclerosis 15, with or without frontotemporal dementia (ALS15) [MIM:300857]: A neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases. Patients with ALS15 may develop frontotemporal dementia.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.11 Ref.12 Ref.13 Ref.14

Sequence similarities: Contains 1 UBA domain.Contains 1 ubiquitin-like domain.
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Research Articles on UBQLN2

1. No causative mutations within the PXX domain of the UBQLN2 gene are found in familial frontotemoral dementia patients.

Precautions

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.

Disclaimer

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.

Product Note

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual, it is intended to serve as an example only. Please refer to the instructions For Use provided with the assay kit for precise details.

Product PubMed Publications

Products associated with anti-UBQLN2 antibody

 Reference Product  PubMed Publications
 UBQLN1 antibody  >10 publications with UBQLN2 and UBQLN1
 EPS15 antibody  >1 publications with UBQLN2 and EPS15
 RAD23A antibody  >1 publications with UBQLN2 and RAD23A
 PSMD4 antibody  >1 publications with UBQLN2 and PSMD4

Pathways associated with anti-UBQLN2 antibody

 Products by Pathway  Pathway Diagram
 Protein Processing In Endoplasmic Reticulum Pathway antibodies  Protein Processing In Endoplasmic Reticulum Pathway Diagram
 Protein Processing In Endoplasmic Reticulum Pathway antibodies  Protein Processing In Endoplasmic Reticulum Pathway Diagram

Diseases associated with anti-UBQLN2 antibody

 Disease Name  Pubmed Publications
 Nervous System Diseases Antibodies  >18 publications with UBQLN2 and Nervous System Diseases
 Brain Diseases Antibodies  >11 publications with UBQLN2 and Brain Diseases
 Drug Toxicity Antibodies  >1 publications with UBQLN2 and Drug Toxicity
 Necrosis Antibodies  >1 publications with UBQLN2 and Necrosis

Organs/Tissues associated with anti-UBQLN2 antibody

 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >5 publications with UBQLN2 and Brain
 Muscle Antibodies  >3 publications with UBQLN2 and Muscle
 Heart Antibodies  >1 publications with UBQLN2 and Heart
 Skin Antibodies  >1 publications with UBQLN2 and Skin
 Blood Antibodies  >1 publications with UBQLN2 and Blood
 Vascular Antibodies  >1 publications with UBQLN2 and Vascular
 Embryonic Tissue Antibodies  >1 publications with UBQLN2 and Embryonic Tissue
 Kidney Antibodies  >1 publications with UBQLN2 and Kidney
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