BW
CLN6 (N258) polyclonal Antibody | BS3102
- SKU:
- BW-BS3102
- Availability:
- Usually ships in 5 working days
Description
CLN6 (N258) polyclonal Antibody | BS3102 | Gentaur UK, US & Europe Distribution
Host: Rabbit
Reactivity: Human,Mouse,Rat
Application: WB IHC
Application Range: WB: 1:500~1:1000 IHC: 1:50~1:200
Background: CLN6, a 311-amino acid protein, has 7 predicted transmembrane domains and is conserved across vertebrates. The CLN6 protein localizes to the endoplasmic reticulum but contributes to lysosomal function. Mutations in the CLN6 gene cause variant late-onset infantile neuronal ceroid lipofuscinosis (vLINCL), a lysosomal storage disorder marked by progressive mental deterioration and blindness; part of a group of severe inherited neurodegenerative disorders affecting children wherein lysosomes accumulate storage material, causing the death of neurons. CLN6 is one of eight proteins, including CLN1-8, that are associated with NCL.
Storage & Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Specificity: CLN6 (N258) polyclonal Antibody detects endogenous levels of CLN6 protein.
Molecular Weight: ~ 40 kDa
Note: For research use only, not for use in diagnostic procedure.
Alternative Names: Ceroid-lipofuscinosis neuronal protein 6; Protein CLN6; CLN6
Immunogen: Synthetic peptide, corresponding to amino acids 229-274 of Human CLN6.
Conjugate: Unconjugated
Modification: Unmodification
Purification & Purity: The Antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .
Pathway: