CLN8 anticorps
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- Antigène Voir toutes CLN8 Anticorps
- CLN8 (Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8))
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Reactivité
- Humain, Chien
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Hôte
- Lapin
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Clonalité
- Polyclonal
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Conjugué
- Cet anticorp CLN8 est non-conjugé
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Application
- Western Blotting (WB), ELISA
- Purification
- Antibody is purified by peptide affinity chromatography method.
- Immunogène
- Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human CLN8.
- Top Product
- Discover our top product CLN8 Anticorps primaire
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- Indications d'application
- 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.
- Restrictions
- For Research Use only
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- Format
- Lyophilized
- Reconstitution
- Add 50 ?L of distilled water. Final antibody concentration is 1 mg/mL.
- Concentration
- 1 mg/mL
- Buffer
- Antibody is lyophilized in PBS buffer with 2 % sucrose.
- Conseil sur la manipulation
- As with any antibody avoid repeat freeze-thaw cycles.
- Stock
- 4 °C/-20 °C
- Stockage commentaire
- For short periods of storage (days) store at 4 °C. For longer periods of storage, store CLN8 antibody at -20 °C.
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- Antigène
- CLN8 (Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8))
- Autre désignation
- CLN8 (CLN8 Produits)
- Synonymes
- anticorps mnd, anticorps C8orf61, anticorps EPMR, anticorps CLN8, transmembrane ER and ERGIC protein, anticorps ceroid-lipofuscinosis, neuronal 8, anticorps CLN8, anticorps Cln8
- Sujet
- 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.
- Poids moléculaire
- 33 kDa
- ID gène
- 2055
- NCBI Accession
- NP_061764
- UniProt
- Q9UBY8
- Pathways
- Regulation of Cell Size, Dicarboxylic Acid Transport
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