ITN2487
ITN2487
- Catalog: ITN2487
- Gene/Protein: CLN3 BTS
- Product Description: Immunotag™ CLN3 Polyclonal Antibody
385.0000
Price in reward points: 385
| Antibody Specification | |
| Datasheet | 
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| Target Protein | CLN3 |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | WB,ELISA |
| Recommended Dilution | WB 1:500-2000 ELISA 1:5000-20000 |
| Concentration | 1 mg/ml |
| Reactive Species | Human |
| Host Species | Rabbit |
| Immunogen | Synthesized peptide derived from human protein . at AA range: 221-270 |
| Specificity | CLN3 Polyclonal Antibody detects endogenous levels of protein. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
| Form | Liquid in PBS containing 50% glycerol, and 0.02% sodium azide. |
| Gene Name | CLN3 BTS |
| Accession No. | Q13286 Q61124 |
| Description | CLN3, battenin(CLN3) Homo sapiens This gene encodes a protein that is involved in lysosomal function. Mutations in this, as well as other neuronal ceroid-lipofuscinosis (CLN) genes, cause neurodegenerative diseases commonly known as Batten disease or collectively known as neuronal ceroid lipofuscinoses (NCLs). Many alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jul 2008], |
| Cell Pathway/ Category | Lysosome, |
| Protein Expression | Brain,Cerebellum,Epithelium,Lung, |
| Subcellular Localization | Golgi membrane,nucleus,cytoplasm,mitochondrion,lysosome,lysosomal membrane,early endosome,late endosome,autophagosome,endoplasmic reticulum,Golgi apparatus,Golgi stack,trans-Golgi network,plasma membrane, |
| Protein Function | Additional isoforms seem to exist,disease:Defects in CLN3 are the cause of Batten disease [MIM:204200]; also known as juvenile-onset ceroid lipofuscinosis neuronal type 3 (CLN3). Batten disease is a recessively inherited neurodegenerative disorder of childhood characterized by progressive loss of vision, seizures, and psychomotor disturbances. Biochemically, the disease is characterized by lysosomal accumulation of hydrophobic material, mainly ATP synthase subunit C. Clinical onset is usually from 5 to 10 years of age. No treatment is available and Batten disease is usually fatal within a decade. The incidence is estimated at 1/20000 to 1/100000 live birth, making it one of the most common neurodegenerative diseases of childhood.,online information:Neural Ceroid Lipofuscinoses mutation db,online information:Retina International's Scientific Newsletter,PTM:Highly glycosylated.,similarity:Belongs to the battenin family., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |
| Material Safety Data Sheet | |
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