ATXN3L anticorps (AA 251-355) (AbBy Fluor® 647)

N° du produit ABIN1396643

Détail du produit anti-ATXN3L anticorps (AbBy Fluor® 647)

Antigène: ATXN3L (Ataxin 3-Like (ATXN3L))

Épitope: AA 251-355

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp ATXN3L est conjugé à/à la AbBy Fluor® 647

Application: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Réactivité croisée: Humain

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human ATXN3L

Isotype: IgG

Information d'application

Indications d'application: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Agent conservateur: ProClin

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

Stock: -20 °C

Stockage commentaire: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Date de péremption: 12 months

Détails sur ATXN3L

Antigène: ATXN3L (Ataxin 3-Like (ATXN3L))

Autre désignation: ATXN3L (ATXN3L Produits)

Synonymes: anticorps MJDL, anticorps ataxin 3 like, anticorps ATXN3L

Sujet:

Synonyms: ATX3L_HUMAN, ATXN3L, Machado-Joseph disease protein 1-like, MJDL, Putative ataxin-3-like protein.

Background: Defects in ATXN3 are the cause of spinocerebellar ataxia type 3 (SCA3) , also known as Machado-Joseph disease (MJD). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA3 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. The molecular defect in SCA3 is the a CAG repeat expansion in ATXN3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease.

ID gène: 92552