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Antibody Product Overview
| ELK.No | ES4682 |
| Product name | CLN5 rabbit pAb |
| Reactivity | Human;Mouse;Rat |
| Applications | WB;ELISA |
| Other name | CLN5; Ceroid-lipofuscinosis neuronal protein 5; Protein CLN5 |
| Size | 100μL |
| Unit price ($) | 248 |
| Human gene ID | 1203 |
| Human Swiss-Prot | O75503 |
| Source | Rabbit |
| Isotype | IgG |
| Target | CLN5 |
| Fields | >>Lysosome |
| Gene name | CLN5 |
| Protein name | Ceroid-lipofuscinosis neuronal protein 5 |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 211286 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | Q3UMW8 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | |
| Rat gene link | |
| Rat Swiss-Prot | |
| Rat Swiss link | |
| 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. |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Clonality | Polyclonal |
| Dilution | Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | |
| Observed band (KD) | 48kD |
| Background | 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], |
| 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., |
| Subcellular location | [Ceroid-lipofuscinosis neuronal protein 5, secreted form]: Lysosome .; [Ceroid-lipofuscinosis neuronal protein 5]: Membrane ; Single-pass type II membrane protein . An amphipathic anchor region facilitates its association with the membrane. . |
| Expression | Ubiquitous. |
Additional Images
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Western Blot analysis of 293T cells using CLN5 Polyclonal Antibody diluted at 1:1000
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Western blot analysis of CLN5 Antibody. The lane on the right is blocked with the CLN5 peptide.
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Western blot analysis of the lysates from COLO205 cells using CLN5 antibody.
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: AO-06-ES4682-100
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