Ataxin 1 anticorps (AA 645-815)

N° du produit ABIN7193320

Détail du produit anti-Ataxin 1 anticorps

Antigène: Ataxin 1 (ATXN1)

Épitope: AA 645-815

Reactivité: Humain

Hôte: Souris

Clonalité: Monoclonal

Conjugué: Cet anticorp Ataxin 1 est non-conjugé

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

Fonction: ATXN1 Antibody

Purification: Purified antibody

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

Clone: 2B8A2

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 (ATXN1 Produits)

Synonymes: anticorps ATX1, anticorps D6S504E, anticorps SCA1, anticorps ATXN1, anticorps ataxin 1b, anticorps atxn1, anticorps 2900016G23Rik, anticorps Atx1, anticorps C85907, anticorps ENSMUSG00000074917, anticorps Gm10786, anticorps Sca1, anticorps CG4547, anticorps Dmel\\CG4547, anticorps dAtx-1, anticorps dAtx1, anticorps sca1, anticorps ataxin 1, anticorps ataxin 1b, anticorps Ataxin 1, anticorps ATXN1, anticorps atxn1b, anticorps Atxn1, anticorps Atx-1

Sujet:

Description:

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.

Aliases: ATX1, SCA1, D6S504E

Poids moléculaire: 86.9kDa

ID gène: 6310

UniProt: P54253

Pathways: Synaptic Membrane