Overview |
bs-11715R |
CLN8 Polyclonal Antibody |
WB, ELISA, IHC-P, IHC-F, IF(IHC-P), IF(IHC-F), IF(ICC), ICC |
Human, Rat |
Mouse, Dog, Pig, Horse, Rabbit |
Specifications |
Unconjugated |
Rabbit |
KLH conjugated synthetic peptide derived from human CLN8 |
201-286/286 |
Polyclonal |
IgG |
1ug/ul |
Purified by Protein A. |
0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
Target |
2055 |
Q9UBY8 |
Cytoplasm, Cell membrane |
Ceroid-lipofuscinosis, neuronal 8 epilepsy, progressive with mental retardation; Cln8; CLN8_HUMAN; EPMR; Protein CLN8; C8orf61 |
CLN8, a 286 amino acid transmembrane protein, localizes mainly to the endoplasmic reticulum, but also partially to the ER-Golgi intermediate compartment (ERGIC). Mutations in the CLN8 gene cause neuronal ceroid lipofuscinosis 8 and progressive epilepsy with mental retardation (EPMR). Both disorders are forms of neuronal ceroid-lipofuscinose (NCL), a group of progressive neurodegenerative diseases found in children, characterized by failure of psychomotor development, impaired vision, seizures and premature death. The CLN8 protein is one of eight proteins in the CLN family, including CLN1-CLN7, which 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 |
ICC |
1:100-500 |