Application
Immunofluorescence staining of methanol-fixed EOC 20 cells showing cytoplasmic localization.| Product Name | atrophin-1 Antibody (H-70) |
|---|---|
| Antibody Type | Primary Antibodies |
| Modification Notes | Dentatorubral-pallidoluysian atrophy protein, also designated atrophin-1, interacts with several other proteins, including RERE, BAIAP2 and WWP1-3. It is highly expressed in ovary, testis, brain and prostate, but can also be detected in thymus, liver and leukocytes. Defects in ATN1, the gene encoding for the atrophin protein, can cause dentatorubral-pallidoluysian atrophy (DRPLA) or Haw River syndrome (HRS). Both disorders are dominant neurodegenerative disorders caused by an increase in the number of polyglutamine (Gln) repeats in the ATN1 gene (7-23 repeats in the normal population, 49-75 in patients affected by DRPLA or HRS). More repeats corresponds to earlier onset and more severe clinical manifestations of the diseases. DRPLA is characterized by a loss of neurons in the dentate nucleus, rubrum, globus pallidus and Luys’ body, often resulting in dementia, epilepsy and cerebellar ataxia. HRS is characterized by the degeneration of multiple systems, resembling that of DRPLA or Huntington’s disease. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of atrophin-1 of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine and porcine | |
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 19-88 mapping near the N-terminus of atrophin-1 of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 19-88 mapping near the N-terminus of atrophin-1 of human origin |
| Entrez Gene | 1822 |
|---|
Application
Immunofluorescence staining of methanol-fixed EOC 20 cells showing cytoplasmic localization.| Application Notes | recommended for detection of atrophin-1 of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine and porcine |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
