UBQLN2 monoclonal antibody (M03), clone 5F5
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More Files
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Western Blot (Cell lysate)
UBQLN2 monoclonal antibody (M03), clone 5F5 Western Blot analysis of UBQLN2 expression in A-431 ( Cat # L015V1 ).
Western Blot (Cell lysate)
UBQLN2 monoclonal antibody (M03), clone 5F5. Western Blot analysis of UBQLN2 expression in NIH/3T3 ( Cat # L018V1 ).
Western Blot (Cell lysate)
UBQLN2 monoclonal antibody (M03), clone 5F5. Western Blot analysis of UBQLN2 expression in Raw 264.7 ( Cat # L024V1 ).
Western Blot (Cell lysate)
UBQLN2 monoclonal antibody (M03), clone 5F5. Western Blot analysis of UBQLN2 expression in PC-12 ( Cat # L012V1 ).
Sandwich ELISA (Recombinant protein)
Detection limit for recombinant GST tagged UBQLN2 is approximately 0.03ng/ml as a capture antibody.
Immunofluorescence
Immunofluorescence of monoclonal antibody to UBQLN2 on A-431 cell. [antibody concentration 10 ug/ml]
Western Blot detection against Immunogen (33.44 KDa) .
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Specification
Product Description
Mouse monoclonal antibody raised against a partial recombinant UBQLN2.
Immunogen
UBQLN2 (NP_038472, 555 a.a. ~ 624 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
PNQQFIQQMVQALAGANAPQLPNPEVRFQQQLEQLNAMGFLNREANLQALIATGGDINAAIERLLGSQPS
Host
Mouse
Reactivity
Human, Mouse, Rat
Interspecies Antigen Sequence
Mouse (87); Rat (86)
Isotype
IgG2a Kappa
Quality Control Testing
Antibody Reactive Against Recombinant Protein.
Western Blot detection against Immunogen (33.44 KDa) .
Storage Buffer
In 1x PBS, pH 7.4
Storage Instruction
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
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Applications
Western Blot (Cell lysate)
UBQLN2 monoclonal antibody (M03), clone 5F5 Western Blot analysis of UBQLN2 expression in A-431 ( Cat # L015V1 ).Western Blot (Cell lysate)
UBQLN2 monoclonal antibody (M03), clone 5F5. Western Blot analysis of UBQLN2 expression in NIH/3T3 ( Cat # L018V1 ).Western Blot (Cell lysate)
UBQLN2 monoclonal antibody (M03), clone 5F5. Western Blot analysis of UBQLN2 expression in Raw 264.7 ( Cat # L024V1 ).Western Blot (Cell lysate)
UBQLN2 monoclonal antibody (M03), clone 5F5. Western Blot analysis of UBQLN2 expression in PC-12 ( Cat # L012V1 ).Western Blot (Recombinant protein)
Sandwich ELISA (Recombinant protein)
Detection limit for recombinant GST tagged UBQLN2 is approximately 0.03ng/ml as a capture antibody.ELISA
Immunofluorescence
Immunofluorescence of monoclonal antibody to UBQLN2 on A-431 cell. [antibody concentration 10 ug/ml] -
Gene Info — UBQLN2
Entrez GeneID
29978GeneBank Accession#
NM_013444Protein Accession#
NP_038472Gene Name
UBQLN2
Gene Alias
CHAP1, CHAP1/DSK2, Dsk2, HRIHFB2157, LIC-2, N4BP4, PLIC-2, PLIC2, RIHFB2157
Gene Description
ubiquilin 2
Omim ID
300264Gene Ontology
HyperlinkGene Summary
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 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
Other Designations
Nedd4 binding protein 4|bA431N15.1 (ubiquilin 2)
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Interactome
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Publication Reference
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Amyotrophic lateral sclerosis-linked UBQLN2 mutants inhibit endoplasmic reticulum to Golgi transport, leading to Golgi fragmentation and ER stress.
Halloran M, Ragagnin AMG, Vidal M, Parakh S, Yang S, Heng B, Grima N, Shahheydari H, Soo KY, Blair I, Guillemin GJ, Sundaramoorthy V, Atkin JD.
Cellular and Molecular Life Sciences : CMLS 2019 Dec; [Epub].
Application：ICC, IF, Mouse, Neuro-2a cells.
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Mutant UBQLN2P497H in motor neurons leads to ALS-like phenotypes and defective autophagy in rats.
Chen T, Huang B, Shi X, Gao L, Huang C.
Acta Neuropathologica Communications 2018 Nov; 6(1):122.
Application：IF, WB-Ti, Rat, Spinal cord.
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Pathologic Involvement of Glutamatergic Striatal Inputs From the Cortices in TAR DNA-Binding Protein 43 kDa-Related Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis.
Riku Y, Watanabe H, Yoshida M, Mimuro M, Iwasaki Y, Masuda M, Ishigaki S, Katsuno M, Sobue G.
Journal of Neuropathology and Eexperimental Neurology 2017 Sep; 76(9):759.
Application：IHC-P, Human, Brain tissue of adult-onset FTLD-TDP patients.
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USP5/Leon deubiquitinase confines postsynaptic growth by maintaining ubiquitin homeostasis through Ubiquilin.
Wang CH, Huang YC, Chen PY, Cheng YJ, Kao HH, Pi H, Chien CT.
Elife 2017 May; 6:e26886.
Application：IF, WB, Larvae, A3 segment of late third instar larvae.
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Marked Involvement of the Striatal Efferent System in TAR DNA-Binding Protein 43 kDa-Related Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis.
Riku Y, Watanabe H, Yoshida M, Mimuro M, Iwasaki Y, Masuda M, Ishigaki S, Katsuno M, Sobue G.
Journal of Neuropathology and Experimental Neurology 2016 Aug; 75(8):801.
Application：IF, Human, Neuron.
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Novel eosinophilic neuronal cytoplasmic inclusions in the external cuneate nucleus of humans.
Ito M, Nakamura K, Mori F, Miki Y, Tanji K, Wakabayashi K.
Neuropathology 2016 Oct; 36(5):441.
Application：IHC-P, Human , Human postmortem specimens.
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Differential recruitment of UBQLN2 to nuclear inclusions in the polyglutamine diseases HD and SCA3.
Zeng L, Wang B, Merillat SA, N Minakawa E, Perkins MD, Ramani B, Tallaksen-Greene SJ, Costa MD, Albin RL, Paulson HL.
Neurobiology of Disease 2015 Oct; 82:281.
Application：IF, Mouse, Hippocampus.
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Drosha Inclusions Are New Components of Dipeptide-Repeat Protein Aggregates in FTLD-TDP and ALS C9orf72 Expansion Cases.
Porta S, Kwong LK, Trojanowski JQ, Lee VM.
Journal of Neuropathology and Experimental Neurology 2015 Apr; 74(4):380.
Application：IF, Human, Brain.
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Pathogenic UBQLN2 gains toxic proterties to induce neuron death.
Wu Q, Liu M, Huang C, Liu X, Huang B, Li N, Zhou H, Xia XG.
Acta Neuropathologica 2015 Mar; 129(3):417.
Application：IF, WB-Tr, Human, HEK 293 cells.
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C9ORF72 repeat-associated non-ATG-translated polypeptides are distributed independently of TDP-43 in a Japanese patient with c9ALS.
Konno T, Tada M, Shiga A, Tsujino A, Eguchi H, Masuda-Suzukake M, Hasegawa M, Nishizawa M, Onodera O, Kakita A, Takahashi H.
Neuropathology and Applied Neurobiology 2014 Oct; 40(6):783.
Application：IHC, Human, Neurones.
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C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal trafficking.
Farg MA, Sundaramoorthy V, Sultana JM, Yang S, Atkinson RA, Levina V, Halloran MA, Gleeson PA, Blair IP, Soo KY, King AE, Atkin JD.
Human Molecular Genetics 2014 Jul; 23(13):3579.
Application：WB, Mouse, Neuro2a cells.
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Lower Motor Neuron Involvement in TAR DNA-Binding Protein of 43 kDa-Related Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis.
Riku Y, Watanabe H, Yoshida M, Tatsumi S, Mimuro M, Iwasaki Y, Katsuno M, Iguchi Y, Masuda M, Senda J, Ishigaki S, Udagawa T, Sobue G.
JAMA Neurology 2014 Feb; 71(2):172.
Application：IHC-P, Human, Nervous.
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Progressive amnestic dementia, hippocampal sclerosis, and mutation in C9ORF72.
Murray ME, Bieniek KF, Banks Greenberg M, Dejesus-Hernandez M, Rutherford NJ, van Blitterswijk M, Niemantsverdriet E, Ash PE, Gendron TF, Kouri N, Baker M, Goodman IJ, Petrucelli L, Rademakers R, Dickson DW.
Acta Neuropathologica 2013 Oct; 126(4):545.
Application：IHC-P, Human, Human brains.
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Ubiquilin-1 immunoreactivity is concentrated on Hirano bodies and dystrophic neurites in Alzheimer's disease brains.
Satoh J, Tabunoki H, Ishida T, Saito Y, Arima K.
Neuropathology and Applied Neurobiology 2013 Dec; 39(7):817.
Application：WB, Human, HEK 293T cells.
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Tau pathology in frontotemporal lobar degeneration with C9ORF72 hexanucleotide repeat expansion.
Bieniek KF, Murray ME, Rutherford NJ, Castanedes-Casey M, DeJesus-Hernandez M, Liesinger AM, Baker MC, Boylan KB, Rademakers R, Dickson DW.
Acta Neuropathologica 2013 Feb; 125(2):289.
Application：IF, IHC-P, Human, Cerebellum, Hippocampus.
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Pattern of ubiquilin pathology in ALS and FTLD indicates presence of C9ORF72 hexanucleotide expansion.
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.
Acta Neuropathologica 2012 Jun; 123(6):825.
Application：IHC, IF, Human, Hippocampal, Cerebellar.
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p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND/ALS.
Al-Sarraj S, King A, Troakes C, Smith B, Maekawa S, Bodi I, Rogelj B, Al-Chalabi A, Hortobagyi T, Shaw CE.
Acta Neuropathologica 2011 Dec; 122(6):691.
Application：IF, Human, Brain.
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Amyotrophic lateral sclerosis-linked UBQLN2 mutants inhibit endoplasmic reticulum to Golgi transport, leading to Golgi fragmentation and ER stress.
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