SAB2104138

Anti-ATXN7 antibody produced in rabbit

affinity isolated antibody

• Synonyme(s) : Anti-ADCAII, Anti-OPCA3, Anti-SCA7
• Code UNSPSC : 12352203
• Nomenclature NACRES : NA.41

Propriétés

• Source biologique: rabbit
• Niveau de qualité: 100
• Conjugué: unconjugated
• Forme d'anticorps: affinity isolated antibody
• Type de produit anticorps: primary antibodies
• Clone: polyclonal
• Forme: buffered aqueous solution
• Poids mol.: 95 kDa
• Espèces réactives: human
• Concentration: 0.5 mg - 1 mg/mL
• Technique(s): western blot: suitable
• Numéro d'accès NCBI: NM_000333
• Numéro d'accès UniProt: O15265
• Conditions d'expédition: wet ice
• Température de stockage: −20°C
• Modification post-traductionnelle de la cible: unmodified
• Informations sur le gène: human ... ATXN7(6314)

Description

Immunogène

Synthetic peptide directed towards the middle region of human ATXN7

Actions biochimiques/physiologiques

ATXN7 is involved in neurodegeneration. ATXN7 acts as component of the STAGA transcription coactivator-HAT complex. ATXN7 mediates the interaction of STAGA complex with the CRX and is involved in CRX-dependent gene activation.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. This locus has been mapped to chromosome 3, and it has been determined that the diseased allele associated with Spinocerebellar ataxia-7, contains 38-130 CAG repeats (near the N-terminus), compared to 7-17 in the normal allele. The exact function of this gene is not known, however, since the encoded protein contains a nuclear localization sequence, and is found to be localized in the nucleus, it has been postulated to be a potential transcription factor. Alternative splicing, resulting in transcript variants encoding different isoforms, has been noted for this gene.

Séquence

Synthetic peptide located within the following region: TRSLTCKTHSLTQRRAVQGRRKRFDVLLAEHKNKTREKELIRHPDSQQPP

Forme physique

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

Clause de non-responsabilité

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Informations sur la sécurité

• Code de la classe de stockage: 10 - Combustible liquids
• Classe de danger pour l'eau (WGK): WGK 3
• Point d'éclair (°F): Not applicable
• Point d'éclair (°C): Not applicable