Target:CLN6
Gene Name:CLN6
Protein Name:Ceroid-lipofuscinosis neuronal protein 6
Human Gene Id:54982
Human Swiss Prot No:Q9NWW5
Immunogen:The antiserum was produced against synthesized peptide derived from human CLN6. AA range:221-270
Specificity:CLN6 Polyclonal Antibody detects endogenous levels of CLN6 protein.
Formulation:Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Source:Polyclonal, Rabbit,IgG
Dilution:WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:40000.. IF 1:50-200
Purification:The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration:1 mg/ml
Storage Stability:-15°C to -25°C/1 year(Do not lower than -25°C)
Other Name:CLN6;Ceroid-lipofuscinosis neuronal protein 6;Protein CLN6
Observed Band(KD):40kD
Background: This gene is one of eight which have been associated with neuronal ceroid lipofuscinoses (NCL). Also referred to as Batten disease, NCL comprises a class of autosomal recessive, neurodegenerative disorders affecting children. The genes responsible likely encode proteins involved in the degradation of post-translationally modified proteins in lysosomes. The primary defect in NCL disorders is thought to be associated with lysosomal storage function. [provided by RefSeq, Oct 2008],
Function:disease:Defects in CLN6 are the cause of variant late-onset infantile neuronal ceroid lipofuscinosis (vLINCL) [MIM:601780].,online information:Neural Ceroid Lipofuscinoses mutation db,
Subcellular Location:Endoplasmic reticulum membrane ; Multi-pass membrane protein . Endoplasmic reticulum .
Expression: Epithelium,Lung,Urinary bladder,