Molecular weight (DA)
40kD
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.
Dilution rate
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/40000.. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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,
Protein name
Ceroid-lipofuscinosis neuronal protein 6
Other name
CLN6; Ceroid-lipofuscinosis neuronal protein 6; Protein CLN6
Human protein sequence Database
Q9NWW5
Mouse protein sequence database
Rat protein sequence database
Cellular localization
Endoplasmic reticulum membrane ; Multi-pass membrane protein . Endoplasmic reticulum .
Tissue expression
Epithelium,Lung,Urinary bladder,
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