Source: | Rabbit | Gene Id: | |
Isotype: | IgG | Swiss Prot: | |
purity: | Purified by Protein A. |
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. CLN6 is one of eight proteins, including CLN1-8, that are associated with NCL. |
Reactivity | Human, Mouse, Rat |
Tested applications | WB IHC IF |
Clonality | Polyclonal Antibody |
Calculated MW | / |
Recommended Dilutions |
WB 1:100-1:1000
IHC 1:100-1:500
IF 1:50-1:200
|
Immunogen | KLH conjugated synthetic peptide derived from human CLN6 |
Storage | Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at -20℃ for 12 months |
Synonym | Ceroid lipofuscinosis, neuronal 6, late infantile, variant, CLN6 protein, FLJ20561, Nclf, Protein CLN6, CLN6_HUMAN. |
沪ICP备15003525号-1 Copyright ©, 2013-2022, Yihyson All Rights Reserved.