Ataxin 3 anticorps

N° du produit ABIN2666310

Détail du produit anti-Ataxin 3 anticorps

Antigène: Ataxin 3 (ATXN3)

Reactivité: Humain

Hôte: Souris

Clonalité: Monoclonal

Conjugué: Cet anticorp Ataxin 3 est non-conjugé

Application: Western Blotting (WB)

Purification: The antibody was purified by affinity chromatography.

Clone: 1H9-2

Isotype: IgG1 kappa

Information d'application

Indications d'application: Optimal working dilution should be determined by the investigator.

Restrictions: For Research Use only

Stockage

Concentration: 0.5 mg/mL

Buffer: phosphate-buffered solution, pH 7.2, containing 0.05 % sodium azide.

Agent conservateur: Sodium azide

Précaution d'utilisation: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Stock: 4 °C

Stockage commentaire: Upon receipt, store undiluted between 2°C and 8°C.

Détails sur Ataxin 3

Antigène: Ataxin 3 (ATXN3)

Autre désignation: Ataxin-3 (ATXN3 Produits)

Synonymes: anticorps Mjd, anticorps zgc:56323, anticorps ATXN3, anticorps at3, anticorps jos, anticorps mjd, anticorps atx3, anticorps mjd1, anticorps sca3, anticorps ataxin-3, anticorps MJD1, anticorps MGC83584, anticorps AT3, anticorps ATX3, anticorps JOS, anticorps MJD, anticorps SCA3, anticorps 2210008M02Rik, anticorps AI463012, anticorps AI647473, anticorps Sca3, anticorps Rsca3, anticorps ataxin 3, anticorps ataxin 3 L homeolog, anticorps ataxin-3, anticorps atxn3, anticorps ATXN3, anticorps atxn3.L, anticorps MICPUN_104046, anticorps MICPUCDRAFT_46658, anticorps Atxn3

Sujet: Ataxin-3 is an ubiquitously expressed protein which contains a poly-glutamine tract. Genetically abnormal expansion of the poly-glutamine repeats results in accumulation of ubiquitinated ataxin-3 protein aggregates and leads to a neurodegenerative disorder, Machado-Joseph disease (MJD). The protein has ubiquitin interacting motifs and a N-terminal Cysteine residue (C14) responsible for its deubiquitinating activity. Ataxin-3 binds and hydrolyzes polyubiquitinated proteins, and interacts with key proteasome components, such as p97/VCP and p45, indicating that Ataxin-3 may play functional roles in ubiquitin-proteasome system. The protein also act as a transcriptional repressor when interacting with transcriptional activators and coactivators.