H00029978-M03

antibody from Novus Biologicals

Targeting: UBQLN2 Chap1, CHAP1/DSK2, Dsk2, LIC-2, N4BP4, PLIC-2, PLIC2, RIHFB2157

Western blot (Supportive data in Antibodypedia)

ELISA (Supportive data in Antibodypedia)

Immunocytochemistry (Supportive data in Antibodypedia)

Immunohistochemistry (Data presented on provider website)

Antibody Data

Product number

H00029978-M03

Provider

Novus Biologicals

Proper citation

Novus Cat#H00029978-M03, RRID:AB_2272420

Product name

Mouse Monoclonal Ubiquilin 2 Antibody

Antibody type

Monoclonal

Description

IgG purified. UBQLN2 (5F5)

Reactivity

Human, Mouse, Rat

Host

Mouse

Isotype

IgG

Vial size

0.1 mg

Storage

Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles.

References

Submitted references

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 4;

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Mutant UBQLN2^P497H 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 8;6(1):122

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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 experimental neurology 2017 Sep 1;76(9):759-768

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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 10;6

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Functional interaction between Lypd6 and nicotinic acetylcholine receptors.

Arvaniti M, Jensen MM, Soni N, Wang H, Klein AB, Thiriet N, Pinborg LH, Muldoon PP, Wienecke J, Imad Damaj M, Kohlmeier KA, Gondré-Lewis MC, Mikkelsen JD, Thomsen MS
Journal of neurochemistry 2016 Sep;138(6):806-20

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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 : official journal of the Japanese Society of Neuropathology 2016 Oct;36(5):441-447

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Ubiquilin2 as a novel marker for detection of urothelial carcinoma cells in urine.

Shimada K, Fujii T, Tatsumi Y, Anai S, Fujimoto K, Konishi N
Diagnostic cytopathology 2016 Jan;44(1):3-9

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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):801-811

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Proteins with Intrinsically Disordered Domains Are Preferentially Recruited to Polyglutamine Aggregates.

Wear MP, Kryndushkin D, O'Meally R, Sonnenberg JL, Cole RN, Shewmaker FP
PloS one 2015;10(8):e0136362

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Differential recruitment of UBQLN2 to nuclear inclusions in the polyglutamine diseases HD and SCA3.

Zeng L, Wang B, Merillat SA, Minakawa EN, Perkins MD, Ramani B, Tallaksen-Greene SJ, Costa MDC, Albin RL, Paulson HL
Neurobiology of disease 2015 Oct;82:281-288

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Pathogenic Ubqln2 gains toxic properties 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):417-28

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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):380-7

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Ubiquilin-1 overexpression increases the lifespan and delays accumulation of Huntingtin aggregates in the R6/2 mouse model of Huntington's disease.

Safren N, El Ayadi A, Chang L, Terrillion CE, Gould TD, Boehning DF, Monteiro MJ
PloS one 2014;9(1):e87513

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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):783-8

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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 1;23(13):3579-95

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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):172-9

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Clinicopathologic report of ocular involvement in ALS patients with C9orf72 mutation.

Fawzi AA, Simonett JM, Purta P, Moss HE, Lowry JL, Deng HX, Siddique N, Sufit R, Bigio EH, Volpe NJ, Siddique T
Amyotrophic lateral sclerosis & frontotemporal degeneration 2014 Dec;15(7-8):569-80

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Ubiquilin 2 is not associated with tau pathology.

Nölle A, van Haastert ES, Zwart R, Hoozemans JJ, Scheper W
PloS one 2013;8(9):e76598

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Sporadic ALS with compound heterozygous mutations in the SQSTM1 gene.

Shimizu H, Toyoshima Y, Shiga A, Yokoseki A, Arakawa K, Sekine Y, Shimohata T, Ikeuchi T, Nishizawa M, Kakita A, Onodera O, Takahashi H
Acta neuropathologica 2013 Sep;126(3):453-9

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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):545-54

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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):289-302

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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):817-30

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An early onset progressive motor neuron disorder in Scyl1-deficient mice is associated with mislocalization of TDP-43.

Pelletier S, Gingras S, Howell S, Vogel P, Ihle JN
The Journal of neuroscience : the official journal of the Society for Neuroscience 2012 Nov 21;32(47):16560-73

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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):825-39

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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, Hortobágyi T, Shaw CE
Acta neuropathologica 2011 Dec;122(6):691-702

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Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia.

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, Ajroud K, Sufit RL, Haines JL, Mugnaini E, Pericak-Vance MA, Siddique T
Nature 2011 Aug 21;477(7363):211-5

Western blot

Supportive validation

Submitted by

Novus Biologicals (provider)

Main image

Experimental details

Western Blot: Ubiquilin 2 Antibody (5F5) [H00029978-M03] - Analysis of UBQLN2 expression in A-431 (Cat # L015V1).

Supportive validation

Submitted by

Novus Biologicals (provider)

Main image

Experimental details

Western Blot: Ubiquilin 2 Antibody (5F5) [H00029978-M03] - Analysis of UBQLN2 expression in NIH/3T3 (Cat # L018V1).

Supportive validation

Submitted by

Novus Biologicals (provider)

Main image

Experimental details

Western Blot: Ubiquilin 2 Antibody (5F5) [H00029978-M03] - UBQLN2 shRNA knockdown in SH-SY5Y cells. First lane is the ladder, then treatment with scrambled shRNA and third lane is UBQLN2 knockdown. 30 ug of the protein sample was loaded on the gel and transferred to the nitrocellulose membrane. Overnight incubation in primary antibodies at 4 °C in 5 % milk in TBST. Then incubated in secondary antibodies (Goat Anti-mouse DyLight 550) at RT for 2 h, and then imaged. Knockdown of the upper band with shRNA for UBQLN2 was about 82 %, and of the lower band only about 8 %, suggesting a high specificity of the used shRNA. This image was submitted via customer Review.

ELISA

Supportive validation

Submitted by

Novus Biologicals (provider)

Main image

Experimental details

ELISA: Ubiquilin 2 Antibody (5F5) [H00029978-M03] - Detection limit for recombinant GST tagged UBQLN2 is approximately 0.03ng/ml as a capture antibody.

Immunocytochemistry

Supportive validation

Submitted by

Novus Biologicals (provider)

Main image

Experimental details

Immunocytochemistry/Immunofluorescence: Ubiquilin 2 Antibody (5F5) [H00029978-M03] - Analysis of monoclonal antibody to UBQLN2 on A-431 cell. Antibody concentration 10 ug/ml