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

Details for Product No. ABIN393265
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Antigen
Synonyms zgc:92244, GB12798, MGC80041, bts, AI323623, BTS, JNCL
Epitope
AA 1-439
(2), (1)
Reactivity
Human
(21), (15), (14), (11)
Host
Mouse
(19), (2)
Clonality (Clone)
Monoclonal ()
Conjugate
Un-conjugated
(1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Application
Western Blotting (WB)
(12), (9), (6), (4), (2), (2), (1)
Pubmed 5 references available
Quantity 0.1 mg
Options
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Catalog No. ABIN393265
450.00 $
Plus shipping costs $45.00

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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
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
anti-Ceroid-Lipofuscinosis, Neuronal 3 (CLN3) (AA 1-439) antibody Western Blot analysis of CLN3 expression in transfected 293T cell line by CLN3 monoclonal antibody (M03), clone 1G10. Lane 1: CLN3 transfected lysate(47.623 KDa). Lane 2: Non-transfected lysate.
Background publications 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).

Sarpong, Schottmann, Rüther et al.: "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).

Imielinski, Baldassano, Griffiths et al.: "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).

Vitiello, Benedict, Padilla-Lopez et al.: "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).

Adams, Beck, Levy et al.: "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).

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