Western blot
0.2-1 ug/mlELISA Titer: 1:12500Positive Control: Hela cell lysate| Product Name | Rabbit anti-CLN8 polyclonal antibody - N-terminal region |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | C8orf61, EPMR, FLJ39417 |
| Immunogen | The immunogen for anti-CLN8 antibody: synthetic peptide directed towards the N terminal of human CLN8 |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WB |
| WB:1:500~1:2000 Notes:Optimal dilutions/concentrations should be determined by the researcher. |
|
| Species Reactivity | DogHorseHuman |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Gene Symbol | CLN8 |
|---|---|
| Gene Synonyms | C8orf61 EPMR FLJ39417 |
| Gene Full Name | Ceroid-lipofuscinosis, neuronal 8 (epilepsy, progressive with mental retardation) |
| Gene Summary | 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. |
| Alternative Names | C8orf61 EPMR FLJ39417 |
| Molecular Weight(MW) | 33kDa |
| Sequence | 286 amino acids |
Western blot
0.2-1 ug/mlELISA Titer: 1:12500Positive Control: Hela cell lysate| Application Notes | WB:1:500~1:2000 Notes:Optimal dilutions/concentrations should be determined by the researcher. |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | Aliquot and store at -20°C. Avoid repeated freeze / thaw cycles |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
