Anti-ATXN7 antibody produced in rabbit
SIGMA/SAB2104138 - affinity isolated antibody
Synonym: Anti-ADCAII; Anti-OPCA3; Anti-SCA7
Product Type: Chemical
Immunoblotting Anti-ATXN7, antibody produced in rabbit: Cat. No. SAB2104138: Immunoblotting analysis of ATXN7 in ACHN cell lysate with ATXN7 anitbody at 1 μg/mL.
| antibody form | affinity isolated antibody |
| antibody product type | primary antibodies |
| biological source | rabbit |
| clone | polyclonal |
| concentration | 0.5 mg - 1 mg/mL |
| conjugate | unconjugated |
| form | buffered aqueous solution |
| mol wt | 95 kDa |
| NCBI accession no. | NM_000333   |
| Quality Level | 100 |
| shipped in | wet ice |
| species reactivity | human |
| storage temp. | −20°C |
| target post-translational modification | unmodified |
| technique(s) | western blot: suitable |
| UniProt accession no. | O15265 |
| Biochem/physiol Actions: | 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. |
| Disclaimer: | 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. |
| Immunogen: | Synthetic peptide directed towards the middle region of human ATXN7 |
| Other Notes: | Synthetic peptide located within the following region: TRSLTCKTHSLTQRRAVQGRRKRFD |
| Physical form: | Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose. |
| RIDADR | NONH for all modes of transport |
| WGK Germany | WGK 3 |
| Flash Point(F) | Not applicable |
| Flash Point(C) | Not applicable |
| Storage Temp. | −20°C |
| UNSPSC | 12352203 |