NHLRC1 Antikörper

Produktnummer ABIN1387027

Dieser Kaninchen Polyklonal Antikörper erkennt spezifisch NHLRC1 in WB, IF (p) und IHC (p). Er zeigt eine Reaktivität gegenüber Human, Ratte und Maus.

Kurzübersicht für NHLRC1 Antikörper (ABIN1387027)

Target: NHLRC1 (NHL Repeat Containing 1 (NHLRC1))

Reaktivität: Human, Ratte, Maus

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser NHLRC1 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Produktdetails anti-NHLRC1 Antikörper

Kreuzreaktivität: Human, Maus, Ratte

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human NHLRC1

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: WB 1:300-5000
IHC-P 1:200-400
IF(IHC-P) 1:50-200

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.

Konservierungsmittel: ProClin

Vorsichtsmaßnahmen: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Haltbarkeit: 12 months

Details zu NHLRC1

Target: NHLRC1 (NHL Repeat Containing 1 (NHLRC1))

Andere Bezeichnung: NHLRC1

Hintergrund:

Synonyms: E3 ubiquitin-protein ligase NHLRC1, EPM2A, EPM2B, Malin, MGC119262, MGC119264, NHL repeat containing 1, NHL repeat containing protein 1, NHL repeat-containing protein 1, NHLC1_HUMAN, NHLRC 1, Nhlrc1.

Background: Progressive myoclonic epilepsy type 2 (EPM2), also called Lafora disease, is an autosomal recessive disease characterized by grand mal seizures and/or myoclonus at about 15 years of age. Rapid and severe mental deterioration follows, often with psychotic features. Survival is less than 10 years after onset. Starch-like, endoplasmic reticulum-associated polyglucosans, called Lafora bodies, can be observed in brain, muscle, liver and heart. One cause of Lafora disease is due to mutations in NHLRC1, the gene encoding Malin. Forty-nine different mutations in NHLRC1 have been shown to cause EPM2. Malin, also called NHL repeat-containing protein 1, is a single subunit E3 ubiquitin ligase, containing 6 NHL repeats and 1 RING-type zinc finger. Malin?s RING domain is responsible for its ability to mediate ubiquitination. Malin interacts with and polyubiquitinates Laforin, a protein also implicated in EPM2. Malin localizes to the endoplasmic reticulum and, to a lesser extent, in the nucleus. Malin is expressed in brain, cerebellum, spinal cord, medulla, heart, liver, skeletal muscle and pancreas.

Gen-ID: 378884