CLN6 Antibody
Cat#:PL0306441
Isotype:Rabbit Polyclonal IgG
Immunogen:Peptide derived from human CLN6.
Purification: Immunogen affinity purified
Storage Buffer: PBS, pH 7.4(1%BSA and 0.1% Sodium azide)
Background:
CLN6, a 311 amino acid protein, has seven 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.
Alternate Names:
Ceroid lipofuscinosis, neuronal 6, late infantile, variant; CLN6 protein; FLJ20561; Nclf; Protein CLN6; CLN6_HUMAN.
Specificity Reaction:Hu, Mo, Rat, Pig, Cow, Hor, , Sheep
Application Notes:WB: 1:100-500 E: 1:500-1000 IP: 1:20-100 IHC: 1:100-500 IF: 1:100-500
Storage :
Store at –20ºC for one year. Avoid repeated freeze-thaw cycles. At 4ºC for 2-3 weeks after reconstituted.
This product for research use only, not for use in humans, therapeutic or diagnostic procedures.
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