CLN6 Polyclonal Antibody
Applications
Predicted Reactivity
| Overview | |
| Catalog # | bs-8017R |
| Product Name | CLN6 Polyclonal Antibody |
| Applications |
WB
ELISA
IHC-P
IHC-F
IF(IHC-P)
IF(IHC-F)
IF(ICC)
|
| Predicted Reactivity | Human, Mouse, Rat, Cow, Sheep, Pig, Horse, Rabbit |
| Specifications | |
| Conjugation | Unconjugated |
| Host | Rabbit |
| Source | KLH conjugated synthetic peptide derived from human CLN6 |
| Immunogen Range | 101-200/311 |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1ug/ul |
| Purification | Purified by Protein A. |
| Storage Buffer | 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Storage Condition | Shipped at 4C. Store at -20C for one year. Avoid repeated freeze/thaw cycles. |
| Target | |
| Gene ID | 54982 |
| Swiss Prot | Q9NWW5 |
| Subcellular location | Cytoplasm, Cell membrane |
| Synonyms | Ceroid lipofuscinosis, neuronal 6, late infantile, variant; CLN6 protein; FLJ20561; Nclf; Protein CLN6; CLN6_HUMAN. |
| 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. |
| Application Dilution | |
| 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 |