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CLN6 (N258) pAb

CLN6 (N258) pAb

Cat no: BS3102


Supplier: Bioworld Technology, Inc.
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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.
Catalogue number: BS3102
Reactivities: Human, Mouse, Rat
Hosts: Rabbit
Applications: Immunohistochemistry, Western Blot
Size: 100ug/100ul
Swiss prot: Q9NWW5
Dilutions: WB: 1:500~1:1000 IHC: 1:50~1:200
Form: liquid
Storage buffer: 1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2.
Concentration: 1ug/ul
Antigen: Synthetic peptide, corresponding to amino acids 229-274 of Human CLN6.
Species: CLN6 (N258) pAb detects endogenous levels of CLN6 protein.
Molecular weight: ~ 40 kDa
Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Storage temp: Store at 4 degrees C short term. Aliquot and store at -20 degrees C long term. Avoid freeze-thaw cycles.
Alternative names: Ceroid-lipofuscinosis neuronal protein 6; Protein CLN6; CLN6
Additional info: For research use only, not for use in diagnostic procedure.

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