CLN6 Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IHC, WB
Recommend Dilution	Western Blot: 1/500-1/1000. Immunohistochemistry on paraffin sections: 1/50-1/200.
Reactivity	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 Condition	Store the antibody undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing.
Predicted Protein Size	~ 40 kDa
Gene Name	ceroid-lipofuscinosis, neuronal 6, late infantile, variant
Database Link	Entrez Gene 54982 Human UniProt ID Q9NWW5
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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