ITT0976
ITT0976
- Catalog: ITT0976
- Gene/Protein: CLN5
- Product Description: Immunotag™ CLN5 Polyclonal Antibody
385.0000
Price in reward points: 385
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Antibody Specification | |
Datasheet | |
Target Protein | CLN5 |
Clonality | Polyclonal |
Storage/Stability | -20°C/1 year |
Application | WB,ELISA |
Recommended Dilution | Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications. |
Concentration | 1 mg/ml |
Reactive Species | Human,Mouse,Rat |
Host Species | Rabbit |
Immunogen | The antiserum was produced against synthesized peptide derived from human CLN5. AA range:171-220 |
Specificity | CLN5 Polyclonal Antibody detects endogenous levels of CLN5 protein. |
Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
Gene Name | CLN5 |
Accession No. | O75503 Q3UMW8 |
Alternate Names | CLN5; Ceroid-lipofuscinosis neuronal protein 5; Protein CLN5 |
Description | ceroid-lipofuscinosis, neuronal 5(CLN5) Homo sapiens This gene is one of eight which have been associated with neuronal ceroid lipofuscinoses (NCL). Also referred to as Batten disease, NCL comprises a class of autosomal recessive, neurodegenerative disorders affecting children. The genes responsible likely encode proteins involved in the degradation of post-translationally modified proteins in lysosomes. The primary defect in NCL disorders is thought to be associated with lysosomal storage function.[provided by RefSeq, Oct 2008], |
Cell Pathway/ Category | Lysosome, |
Protein Expression | Fetal brain, |
Subcellular Localization | lysosome,lysosomal membrane,endoplasmic reticulum,Golgi apparatus,cytosol,integral component of membrane,perinuclear region of cytoplasm,extracellular exosome, |
Protein Function | disease:Defects in CLN5 are the cause of ceroid lipofuscinosis neuronal 5 (CLN5) [MIM:256731]; also known as Finnish variant late-infantile neuronal ceroid lipofuscinosis (vLINCL). It is a fatal childhood neurodegenerative disease characterized by progressive visual and mental decline, motor disturbance, epilepsy and behavioral changes. The first symptom is motor clumsiness, followed by progressive visual failure, mental and motor deterioration and later by myoclonia and seizures.,online information:Neural Ceroid Lipofuscinoses mutation db,PTM:Glycosylated.,similarity:Belongs to the CLN5 family.,tissue specificity:Ubiquitous., |
Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |