CLN5 Polyclonal Antibody
Applications
Reactivity
Predicted Reactivity
| Overview | |
| Catalog # | bs-11714R |
| Product Name | CLN5 Polyclonal Antibody |
| Applications |
WB
ELISA
IHC-P
IHC-F
IF(IHC-P)
IF(IHC-F)
IF(ICC)
|
| Reactivity | Human |
| Predicted Reactivity | Mouse, Rat, Cow, Sheep, Pig, Horse, Rabbit |
| Specifications | |
| Conjugation | Unconjugated |
| Host | Rabbit |
| Source | KLH conjugated synthetic peptide derived from human CLN5 |
| Immunogen Range | 61-120/358 |
| 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 | |
| Subcellular location | Cytoplasm |
| Synonyms | Ceroid lipofuscinosis neuronal 5; Ceroid-lipofuscinosis neuronal protein 5; CLN5; CLN5_HUMAN; NCL; Protein CLN5. |
| Background | Neuronal ceroid-lipofuscinose (NCL), also designated Batten disease, comprises a group of recessively inherited, progressive neurodegenerative diseases found in children. NCL is characterized by atrophy of the brain and an accumulation of lysosome derived fluorescent bodies found in many cells, especially neurons. Symptoms of NCL include a failure of psychomotor development, seizures, impaired vision and premature death. The eight genes/proteins associated with NCL are designated CLN1-CLN8. Mutations in six of these genes results in a distinct type of NCL-disease; the six genes/proteins are CLN1 (encoding PPT1, a protein thiolesterase), CLN2 (encodeing the serine protease TPP1), CLN3, CLN5, CLN6 and CLN8. A single base duplication mutation in dog and cow CLN5 has been shown to cause 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 |