Atrophin-1 rabbit pAb - ES1734

Atrophin-1 rabbit pAb

Sizes: 50μL, 100μL

Catalogue Numbers: ES1734-50, ES1734-100

Citations, Manuals and MSDS Available upon request.

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],

Alternate Name: ATN1; D12S755E; DRPLA; Atrophin-1; Dentatorubral-pallidoluysian atrophy protein

Source: Rabbit

Applications: WB; ELISA

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

Reactivity: Human; Mouse; Rat

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

Storage and Stability: -20°C/1 year

Clonality: Polyclonal

Isotype: IgG

Concentration: 1 mg/ml

Observed Band (KD): 130kD

Human Gene ID: 1822

Human SWISS Prot NO: P54259

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.

Research Use Only