
- Species ReactivityHuman, Mouse
- SpecificityDetects human and mouse ATN1 in Western blots.
- SourcePolyclonal Sheep IgG
- PurificationAntigen Affinity-purified
- ImmunogenE. coli-derived recombinant human ATN1
Met1-Gln100
Accession # P54259 - FormulationLyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied as a 0.2 µm filtered solution in PBS.
- LabelUnconjugated
- Western Blot1 µg/mLSee below
- Immunohistochemistry5-15 µg/mLSee below
- ReconstitutionSterile PBS to a final concentration of 0.2 mg/mL.
- ShippingThe product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below. *Small pack size (SP) is shipped with polar packs. Upon receipt, store it immediately at -20 to -70 °C
- Stability & StorageUse a manual defrost freezer and avoid repeated freeze-thaw cycles.
- 12 months from date of receipt, -20 to -70 °C as supplied.
- 1 month, 2 to 8 °C under sterile conditions after reconstitution.
- 6 months, -20 to -70 °C under sterile conditions after reconstitution.
- Long Name:Atrophin 1
- Entrez Gene IDs:1822 (Human); 13498 (Mouse); 29515 (Rat)
- Alternate Names:ATN1; atrophin 1; atrophin-1; B37; D12S755E; dentatorubral-pallidoluysian atrophy (atrophin-1); Dentatorubral-pallidoluysian atrophy protein; DRPLA; DRPLANOD; HRS; NOD
Background:
ATN1 (Atrophin-1; also DRPLA protein) is a 190‑200 kDa member of the Atrophin family of proteins that is cleaved into 120‑150 kDa fragments. ATN1 is a ubiquitously expressed transcriptional coactivator. Human ATN1 is 1185 amino acids (aa) in length. It contains multiple motifs, including an NLS (aa 16-32), interspersed poly-Pro, poly-Ser and poly-His regions (aa 376-707), two RE (ArgGlu) repeats (aa 816-934), and an NES (aa 1033-1042). There are at least 17 utilized phosphorylation sites and one acetylated Lys. ATN1 is most characterized by a poly-Gln region between aa 484-497. Normally, there are about 20 consecutive Gln residues, but this number may be increased to more than 70 in pathologic conditions. Proteolytic cleavage generates large C-terminal fragments of 120-150 kDa size. These are unlikely to contain the NLS, and thus are typically cytosolic. ATN1 is suggested to form heterodimers with full-length ATN2/RERE, thus generating a transcriptional repressor. There are multiple potential isoforms. One shows an alternative start site at Met527, while others differ in the number of glutamines in the poly-Gln region. Over aa 1-100, human ATN1 shares 94% aa identity with mouse ATN1.
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