CLN6 Rabbit Polyclonal Antibody
Other products for "CLN6"
Specifications
Product Data | |
Applications | IHC, WB |
Recommended Dilution | Western Blot: 1/500-1/1000. Immunohistochemistry on paraffin sections: 1/50-1/200. |
Reactivities | Human |
Host | Rabbit |
Clonality | Polyclonal |
Specificity | CLN6 antibody detects endogenous levels of CLN6 protein. (region surrounding asn258) |
Formulation | Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2. State: Aff - Purified State: Liquid purified Ig fraction |
Concentration | 1.0 mg/ml |
Purification | Affinity chromatography |
Conjugation | Unconjugated |
Storage | Store the antibody undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing. |
Stability | Shelf life: one year from despatch. |
Predicted Protein Size | ~ 40 kDa |
Gene Name | ceroid-lipofuscinosis, neuronal 6, late infantile, variant |
Database Link | |
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. |
Synonyms | FLJ20561; HsT18960; nclf |
Reference Data | |
Protein Families | Transmembrane |
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