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CLN3 antibody (Ceroid-Lipofuscinosis, Neuronal 3) (AA 1-439)

Details for Product anti-CLN3 Antibody No. ABIN393265, Supplier: Log in to see
Antigen
  • zgc:92244
  • GB12798
  • MGC80041
  • bts
  • AI323623
  • BTS
  • JNCL
  • ceroid-lipofuscinosis, neuronal 3
  • battenin
  • ceroid lipofuscinosis, neuronal 3, juvenile (Batten, Spielmeyer-Vogt disease)
  • cln3
  • PTRG_05620
  • CLN3
  • Cln3
Epitope
AA 1-439
15
6
2
2
1
1
Reactivity
Human
36
25
23
Host
Mouse
33
3
Clonality (Clone)
Monoclonal ()
Conjugate
This CLN3 antibody is un-conjugated
1
1
1
1
1
1
1
1
1
1
1
1
1
1
Application
Western Blotting (WB)
22
13
12
8
4
3
1
Options
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Immunogen CLN3 (AAH02394, 1 a.a. ~ 439 a.a) full length recombinant protein with GST tag. MW of the GST tag alone is 26 K
Clone 1G10
Isotype IgG2a kappa
Specificity CLN3 (AAH02394, 1 a.a. ~ 439 a.a) full length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Purification This antibody is purified through a protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS.
Alternative Name CLN3 (CLN3 Antibody Abstract)
Background Synonyms: Serine protease inhibitor J6, 47 kDa heat shock protein, Collagen-binding protein, Colligin, Serpin H1,Hsp47, Cbp1, Serpinh1
Molecular Weight 47623 DA
Gene ID 12406
Research Area Neurology, Cell Structure
Application Notes Western blot = 1:500-1000
Comment

Background: This gene encodes a protein that is involved in lysosomal function. Mutations in this, as well as other neuronal ceroid-lipofuscinosis (CLN) genes, cause neurodegenerative diseases commonly known as Batten disease or collectively known as neuronal ceroid lipofuscinoses (NCLs). Many alternatively spliced transcript variants have been found for this gene.

Restrictions For Research Use only
Concentration 1 mg/ml
Buffer PBS with 0.09% (w/v) sodium azide.
Preservative Sodium azide
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Storage 4 °C/-20 °C
Storage Comment Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles
Expiry Date 6 months
Supplier Images
Western Blotting (WB) image for anti-CLN3 antibody (Ceroid-Lipofuscinosis, Neuronal 3) (AA 1-439) (ABIN393265) Western Blot analysis of CLN3 expression in transfected 293T cell line by CLN3 monocl...
Background publications Adams, Beck, Levy, Jordan, Kwon, Marshall, Vierhile, Augustine, de Blieck, Pearce, Mink: "Genotype does not predict severity of behavioural phenotype in juvenile neuronal ceroid lipofuscinosis (Batten disease)." in: Developmental medicine and child neurology, Vol. 52, Issue 7, pp. 637-43, 2010 (PubMed).

Vitiello, Benedict, Padilla-Lopez, Pearce: "Interaction between Sdo1p and Btn1p in the Saccharomyces cerevisiae model for Batten disease." in: Human molecular genetics, Vol. 19, Issue 5, pp. 931-42, 2010 (PubMed).

Imielinski, Baldassano, Griffiths, Russell, Annese, Dubinsky, Kugathasan, Bradfield, Walters, Sleiman, Kim, Muise, Wang, Glessner, Saeed, Zhang, Frackelton, Hou, Flory, Otieno, Chiavacci, Grundmeier, Castro, Latiano, Dallapiccola, Stempak, Abrams, Taylor,: "Common variants at five new loci associated with early-onset inflammatory bowel disease." in: Nature genetics, Vol. 41, Issue 12, pp. 1335-40, 2009 (PubMed).

Sarpong, Schottmann, Rüther, Stoltenburg, Kohlschütter, Hübner, Schuelke: "Protracted course of juvenile ceroid lipofuscinosis associated with a novel CLN3 mutation (p.Y199X)." in: Clinical genetics, Vol. 76, Issue 1, pp. 38-45, 2009 (PubMed).

Codlin, Mole: "S. pombe btn1, the orthologue of the Batten disease gene CLN3, is required for vacuole protein sorting of Cpy1p and Golgi exit of Vps10p." in: Journal of cell science, Vol. 122, Issue Pt 8, pp. 1163-73, 2009 (PubMed).