| Product Name | Anti-ATXN1 antibody-RBITC |
|---|---|
| Antibody Type | Primary Antibodies |
| Immunogen | Polypeptide |
| Conjugation | RBITC |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Protein A |
| Gene Symbol | ATXN1 |
|---|---|
| Gene Synonyms | 2900016G23Rik ATX1 D6S504E Gm10786 SCA1 |
| Gene Full Name | ataxin 1 |
| Gene Summary | 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. [provided by RefSeq, Nov 2017] |
| Alternative Names | Alt-ATXN1 alternative ataxin1 OTTHUMP00000016065 spinocerebellar ataxia 1 spinocerebellar ataxia 1 homolog spinocerebellar ataxia type 1 protein spinocerebellar ataxia type 1 protein homolog |
| Molecular Weight(MW) | 87kDa（observed：33kDa） |
| Cellular Localization | Cytoplasm, Nucleus |
| SwissProt ID | P54253 |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | Shipped at 4°C. Store at +4°C short term (1-2 weeks). Store at -20°C long term. Avoid freeze / thaw cycle. |
| Storage Buffer | Purified antibody in PBS with 0.05% PC300,40% Glycerol. |
