CLN8 Antikörper

Produktnummer ABIN2459283

Der Kaninchen Polyklonal anti-CLN8 Antikörper (ABIN2459283) detektiert spezifisch CLN8 in WB und ELISA. Dieser Antikörper reagiert spezifisch mit Proben aus Human und Hund.

Kurzübersicht für CLN8 Antikörper (ABIN2459283)

Target: CLN8 (Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8))

Reaktivität: Human, Hund

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser CLN8 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA

Produktdetails anti-CLN8 Antikörper

Aufreinigung: Antibody is purified by peptide affinity chromatography method.

Immunogen: Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human CLN8.

Anwendungsinformationen

Applikationshinweise: 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.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Rekonstitution: Add 50 ?L of distilled water. Final antibody concentration is 1 mg/mL.

Konzentration: 1 mg/mL

Buffer: Antibody is lyophilized in PBS buffer with 2 % sucrose.

Handhabung: As with any antibody avoid repeat freeze-thaw cycles.

Lagerung: 4 °C/-20 °C

Informationen zur Lagerung: For short periods of storage (days) store at 4 °C. For longer periods of storage, store CLN8 antibody at -20 °C.

Details zu CLN8

Target: CLN8 (Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8))

Andere Bezeichnung: CLN8

Hintergrund: 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.

Molekulargewicht: 33 kDa

Gen-ID: 2055

NCBI Accession: NP_061764

UniProt: Q9UBY8

Pathways: Regulation of Cell Size, Dicarboxylic Acid Transport