Invitrogen
Catalog # PA5-50006
Quantity:
TESTED APPLICATIONS | DILUTION |
---|---|
Immunohistochemistry (Paraffin) (IHC (P)) | 1:50-1:100 |
Western Blot (WB) | 1:500-1:3,000 |
Species reactivity | Human |
Host / Isotype | Rabbit / IgG |
Class | Polyclonal |
Type | Antibody |
Immunogen | Synthesized peptide derived from internal of human CLN6. |
Conjugate | Unconjugated |
Form | Liquid |
Concentration | 1 mg/mL |
Purification | Antigen affinity chromatography |
Storage buffer | PBS, pH 7.4, with 50% glycerol |
Contains | 0.02% sodium azide |
Storage conditions | -20°C |
RRID | AB_2635459 |
The antibody detects endogenous levels of total CLN6 protein.
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: ceroid-lipofuscinosis; ceroid-lipofuscinosis neuronal 6 late infantile; Ceroid-lipofuscinosis neuronal protein 6; Protein CLN6
Gene Aliases: CLN4A; CLN6; HsT18960; nclf
UniProt ID: (Human) Q9NWW5
Entrez Gene ID: (Human) 54982
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