bs-8017R

DATASHEET

Host: Rabbit

Target Protein: CLN6

Immunogen Range: 101-200/311

Clonality: Polyclonal

Isotype: IgG

Entrez Gene: 54982

Swiss Prot: Q9NWW5

Source: KLH conjugated synthetic peptide derived from human CLN6

Purification: Purified by Protein A.

Storage Buffer: 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

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.

Size: 100ul

Concentration: 1ug/ul

Applications: WB(1:300-5000)
ELISA(1:500-1000)
IHC-P(1:200-400)
IHC-F(1:100-500)
IF(IHC-P)(1:50-200)
IF(IHC-F)(1:50-200)
IF(ICC)(1:50-200)

Predicted Molecular Weight: 36

Predicted Cross Reactive Species: Human
Mouse
Rat
Cow
Sheep
Pig
Horse
Rabbit

For research use only. Not intended for diagnostic or therapeutic use.