Thermo Fisher Scientific CLN3 Polyclonal Antibody

Applications

Tested Dilution

Publications

Immunohistochemistry (Paraffin) (IHC (P))

1:200-1:500

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

Recombinant protein corresponding to Human CLN3. Recombinant protein control fragment (Product #RP-104248). if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:CLN3,uniProtId:Q13286-1,ncbiNodeId:9606,antigenRange:242-278,antigenLength:438,antigenImageFileName:PA5-64103_CLN3_Q13286-1_Rabbit.svg,antigenImageFileNamePDP:PA5-64103_CLN3_Q13286-1_Rabbit_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

View immunogen .st0{fill:#FFFFFF;} .st1{fill:#1E8AE7;}

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

0.1 mg/mL

Purification

Antigen affinity chromatography

Storage buffer

PBS, pH 7.2, with 40% glycerol

Contains

0.02% sodium azide

Storage conditions

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

Shipping conditions

Wet ice

RRID

AB_2639893

Product Specific Information

Immunogen sequence: EEEAESAARQ PLIRTEAPES KPGSSSSLSL RERWTVF

Highest antigen sequence identity to the following orthologs: Mouse - 78%, Rat - 73%.

Target Information

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.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.