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ELK Biotechnology

SKU:ES1734

Atrophin-1 rabbit pAb

Atrophin-1 rabbit pAb

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Size
Applications: WB;ELISA
Reactivity: Human;Mouse;Rat
Source: Rabbit
Dilution: Western Blot: 1/500 - 1/2000. ELISA: 1/5000. Not yet tested in other applications.
Immunogen: The antiserum was produced against synthesized peptide derived from human ATN1. AA range:81-130
Storage_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. .
Other_name: ATN1; D12S755E; DRPLA; Atrophin-1; Dentatorubral-pallidoluysian atrophy protein
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],
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