Ataxin 1 anticorps (AA 645-815)

N° du produit ABIN7193320

Cet anticorps Souris Monoclonal détecte spécifiquement Ataxin 1 dans WB, ELISA et FACS. Il présente une réactivité envers Humain et Souris.

Aperçu rapide pour Ataxin 1 anticorps (AA 645-815) (ABIN7193320)

Antigène: Ataxin 1 (ATXN1)

Reactivité: Humain, Souris

Hôte: Souris

Clonalité: Monoclonal

Conjugué: Cet anticorp Ataxin 1 est non-conjugé

Application: Western Blotting (WB), ELISA, Flow Cytometry (FACS)

Clone: 2B8A2

Détail du produit anti-Ataxin 1 anticorps

Épitope: AA 645-815

Fonction: ATXN1 Antibody

Purification: Purified antibody

Immunogène: Purified recombinant fragment of human ATXN1 (AA: 645-815) expressed in E. Coli.

Isotype: IgG1

Information d'application

Indications d'application:

ELISA: 1/10000

FCM: 1/200 - 1/400

Restrictions: For Research Use only

Stockage

Format: Liquid

Buffer: Purified antibody in PBS with 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,-20 °C

Stockage commentaire: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.

Détails sur Ataxin 1

Antigène: Ataxin 1 (ATXN1)

Autre désignation: ATXN1

Sujet:

The autosomal dominant cerebellar ataxias (ADCA) are a heterogeneous group of neurodegenerative disorders characterized by progressive degeneration of the cerebellum, brain stem and spinal cord. Clinically, ADCA has been divided into three groups: ADCA types I-III. ADCAI is genetically heterogeneous, with five genetic loci, designated spinocerebellar ataxia (SCA) 1, 2, 3, 4 and 6, being assigned to five different chromosomes. ADCAII, which always presents with retinal degeneration (SCA7), and ADCAIII often referred to as the `pure' cerebellar syndrome (SCA5), are most likely homogeneous disorders. Several SCA genes have been cloned and shown to contain CAG repeats in their coding regions. ADCA is caused by the expansion of the CAG repeats, producing an elongated polyglutamine tract in the corresponding protein. The expanded repeats are variable in size and unstable, usually increasing in size when transmitted to successive generations. The function of the ataxins is not known. This locus has been mapped to chromosome 6, and it has been determined that the diseased allele contains 40-83 CAG repeats, compared to 6-39 in the normal allele, and is associated with spinocerebellar ataxia type 1 (SCA1). Alternative splicing results in multiple transcript variants, with one variant encoding multiple distinct proteins, ATXN1 and Alt-ATXN1, due to the use of overlapping alternate reading frames.

Poids moléculaire: 86.9 kDa

UniProt: P54253

Pathways: Synaptic Membrane