Atrophin-1 rabbit pAb

• Background :

  Dentatorubral pallidoluysian atrophy (DRPLA) is a rare neurodegenerative disorder characterized by cerebellar ataxia, myoclonic epilepsy, choreoathetosis, and dementia. The disorder is related to the expansion from 7-35 copies to 49-93 copies of a trinucleotide repeat (CAG/CAA) within this gene. The encoded protein includes a serine repeat and a region of alternating acidic and basic amino acids, as well as the variable glutamine repeat. Alternative splicing results in two transcripts variants that encode the same protein. [provided by RefSeq, Jul 2016]

• Description :

  Dentatorubral pallidoluysian atrophy (DRPLA) is a rare neurodegenerative disorder characterized by cerebellar ataxia, myoclonic epilepsy, choreoathetosis, and dementia. The disorder is related to the expansion from 7-35 copies to 49-93 copies of a trinucleotide repeat (CAG/CAA) within this gene. The encoded protein includes a serine repeat and a region of alternating acidic and basic amino acids, as well as the variable glutamine repeat. Alternative splicing results in two transcripts variants that encode the same protein. [provided by RefSeq, Jul 2016],

• Product Name Alternative :

  ATN1; D12S755E; DRPLA; Atrophin-1; Dentatorubral-pallidoluysian atrophy protein

• UniProt :

  P54259

• Swiss Prot :

  P54259

• Reactivity :

  Human; Mouse; Rat

• Immunogen :

  The antiserum was produced against synthesized peptide derived from human ATN1. AA range:81-130

• Target :

  Atrophin-1

• Clonality :

  Polyclonal

• Source :

  Rabbit

• Applications :

  WB; ELISA

• Concentration :

  1 mg/ml

• Dilution :

  Western Blot: 1/500 - 1/2000. ELISA: 1/5000. Not yet tested in other applications.

• Buffer :

  -20°C/1 year

• Molecular Weight :

  130kD

• Storage Conditions :

  -20°C/1 year

• Observed Molecular Weight :

  130kD

• Fragment :

  IgG

• Subcellular Location :

  Nucleus. Cytoplasm, perinuclear region. Cell junction . Shuttles between nucleus and cytoplasm. Colocalizes with FAT1 in the perinuclear area, at cell-cell junctions and leading edges of cells (By similarity) . Colocalizes with MTG8 in discrete nuclear dots. Proteolytic fragment F1 appears to remain in nucleus. Fragment F2 is exported into the cytoplasm. Fragment F2 from mutant sequences with longer poly-Gln (polyQ) tracts are additionally located to the cytoplasmic membrane and to certain organelles. .

• Other Product Names :

  ATN1; D12S755E; DRPLA; Atrophin-1; Dentatorubral-pallidoluysian atrophy protein

• Gene ID (Human) :

  1822