Application
Antibody used in WB on Human HeLa at 0.2-1 ug/ml.| Product Name | CLN8 Antibody |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | CLN8, C8orf61, EPMR, FLJ39417 |
| Product description | CLN8 is a transmembrane protein belonging to a family of proteins containing TLC domains, which are postulated to function in lipid synthesis, transport, or sensing. The protein localizes to the endoplasmic reticulum (ER), and may recycle between the ER and ER-Golgi intermediate compartment. Mutations in this gene are associated with progressive epilepsy with mental retardation (EMPR), which is a subtype of neuronal ceroid lipofuscinoses (NCL). Patients with mutations in this gene have altered levels of sphingolipid and phospholipids in the brain. Childhood-onset NCL are a group of autosomal recessive progressive encephalopathies characterized by the accumulation of autofluorescent material, mainly ATP synthase subunit C, in various tissues, notably in neurons. Based on clinical features, the country of origin of patients, and the molecular genetic background of the disorder, at least seven different forms are thought to exist. CLN8 is characterized by normal early development, onset of generalized seizures between 5 and 10 years, and subsequent progressive mental retardation.This gene encodes a transmembrane protein belonging to a family of proteins containing TLC domains, which are postulated to function in lipid synthesis, transport, or sensing. The protein localizes to the endoplasmic reticulum (ER), and may recycle between the ER and ER-Golgi intermediate compartment. Mutations in this gene are associated with progressive epilepsy with mental retardation (EMPR), which is a subtype of neuronal ceroid lipofuscinoses (NCL). Patients with mutations in this gene have altered levels of sphingolipid and phospholipids in the brain.1) Hermansson, M., (2005) J. Neurochem. 95 (3), 609-617. |
| Immunogen | Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human CLN8. |
| Clonality | Polyclonal |
|---|---|
| Host Species | Rabbit |
| Tested Applications | ELISAWB |
| CLN8 antibody can be used for detection of CLN8 by ELISA at 1:12500. CLN8 antibody can be used for detection of CLN8 by western blot at 1 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.: |
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| Species Reactivity | DogHuman |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Gene Symbol | CLN8 |
|---|---|
| Alternative Names | CLN8 C8orf61 EPMR FLJ39417 |
| Molecular Weight(MW) | 33 kDa |
Application
Antibody used in WB on Human HeLa at 0.2-1 ug/ml.| Positive Control | 1) Cat. No. 1201 - HeLa Cell Lysate |
|---|---|
| Application Notes | CLN8 antibody can be used for detection of CLN8 by ELISA at 1:12500. CLN8 antibody can be used for detection of CLN8 by western blot at 1 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.: |
| Form | Liquid |
|---|---|
| Storage Instructions | For short periods of storage (days) store at 4˚C. For longer periods of storage, store CLN8 antibody at -20˚C. As with any antibody avoid repeat freeze-thaw cycles. |
| Storage Buffer | Antibody is lyophilized in PBS buffer with 2% sucrose. Add 50 μL of distilled water. Final antibody concentration is 1 mg/mL. |
