CLN6 Polyclonal Antibody, AbBy Fluor™ 488 Conjugated
Product Specifications
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.
Synonyms
Ceroid lipofuscinosis, neuronal 6, late infantile, variant; CLN6 protein; FLJ20561; Nclf; Protein CLN6; CLN6_HUMAN.
Gene ID
54982
Swiss Prot
Q9NWW5
Cellular Locus
Cytoplasm, Cell membrane
Host
Rabbit
Immunogen
101-200/311
Target
CLN6
Clonality
Polyclonal
Isotype
IgG
Conjugation
AbBy Fluor™ 488
Source
KLH conjugated synthetic peptide derived from human CLN6
Applications
WB, IF (IHC-P), IF (IHC-F), IF (ICC)
Purification
Purified by Protein A.
Excitation Emission
499nm/519nm
Concentration
1µg/µl
Dilution
WB (1:300-5000), IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)
Buffer
Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Modification
Unmodified
Storage Conditions
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Gene ID URL
54982
Predicted Cross Reactivity
Human, Mouse, Rat, Cow, Sheep, Pig, Horse, Rabbit
Curated Selection
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