CLN3 Polyclonal Antibody | Invitrogen PA5-49382 product information

This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.

product summary

company name :

Invitrogen

other brands :

NeoMarkers, Lab Vision, Endogen, Pierce, BioSource International, Zymed Laboratories, Caltag, Molecular Probes, Research Genetics, Life Technologies, Applied Biosystems, GIBCO BRL, ABgene, Dynal, Affinity BioReagents, Nunc, Invitrogen, NatuTec, Oxoid, Richard-Allan Scientific, Arcturus, Perseptive Biosystems, Proxeon, eBioscience

product type :

antibody

product name :

CLN3 Polyclonal Antibody

catalog :

PA5-49382

quantity :

400 uL

price :

US 454.00

clonality :

polyclonal

host :

domestic rabbit

conjugate :

nonconjugated

reactivity :

human, mouse

application :

western blot

citations: 1

Reference
Zhang X, Zhang D, Thompson J, Chen S, Huang Z, Jennings L, et al. Gene correction of the CLN3 c.175G>A variant in patient-derived induced pluripotent stem cells prevents pathological changes in retinal organoids. Mol Genet Genomic Med. 2021;9:e1601 pubmed publisher

product information

Product Type :

Antibody

Product Name :

CLN3 Polyclonal Antibody

Catalog # :

PA5-49382

Quantity :

400 uL

Price :

US 454.00

Clonality :

Polyclonal

Purity :

Antigen affinity chromatography, Protein A

Host :

Rabbit

Reactivity :

Human, Mouse

Applications :

Western Blot: 1:1000

Species :

Human, Mouse

Isotype :

IgG

Storage :

Store at 4 C short term. For long term storage, store at -20 C, avoiding freeze/thaw cycles.

Description :

CLN3 is a highly glycosylated, hydrophobic, 438-amino acid protein with 6 transmembrane domains. The CLN3 protein localizes to the lysosomal membrane and plays a role in lysosomal function. It may act as a chaperone involved in the folding and unfolding of other proteins, namely subunit C of the ATP synthase complex. Mutations in the CLN3 gene cause Batten disease, a recessively inherited neurodegenerative disorder of childhood caused by lysosomal accumulation of hydrophobic material, mainly ATP synthase subunit C. Batten disease is the most common form of a group of disorders known as neuronal ceroid lipofuscinoses (NCLs). Symptoms of Batten disease include progressive loss of vision, seizures, and psychomotor disturbances.

Immunogen :

KLH conjugated synthetic peptide between 250-284 amino acids from the Central region of human CLN3.

Format :

Liquid

Applications w/Dilutions :

Western Blot: 1:1000

Aliases :

AI323623; batten disease protein; Battenin; BTS; ceroid lipofuscinosis, neuronal 3, juvenile (Batten, Spielmeyer-Vogt disease); ceroid-lipofuscinosis, neuronal 3; CLN 3; Cln3; CLN3, battenin; Cln3p; JNCL; MGC102840; Protein CLN3