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Mouse Anti-ATN1 Recombinant Antibody (2C10) (CBMAB-D0031-YC)

Provided herein is a Mouse monoclonal antibody, which binds to Atrophin 1 (ATN1). The antibody can be used for immunoassay techniques, such as ELISA, IF, WB.
See all ATN1 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
2C10
Antibody Isotype
IgG1, κ
Application
ELISA, IF, WB

Basic Information

Immunogen
ATN1 (AAH51795, 1 a.a. ~ 110 a.a) partial recombinant protein with GST tag.
Specificity
Human
Antibody Isotype
IgG1, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
IF(ICC)10 µg/ml

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.4
Preservative
None
Concentration
Batch dependent
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Atrophin 1
Introduction
ATN1 includes a serine repeat and a region of alternating acidic and basic amino acids, as well as the variable glutamine repeat. 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.
Entrez Gene ID
1822
UniProt ID
P54259
Alternative Names
Atrophin 1; Dentatorubral-Pallidoluysian Atrophy Protein; D12S755E; DRPLA; Dentatorubral-Pallidoluysian Atrophy (Atrophin-1); Atrophin-1; B37; HRS; NOD;
Function
Transcriptional corepressor. Recruits NR2E1 to repress transcription. Promotes vascular smooth cell (VSMC) migration and orientation (By similarity).
Corepressor of MTG8 transcriptional repression. Has some intrinsic repression activity which is independent of the number of poly-Gln (polyQ) repeats.
Biological Process
Central nervous system development Source: ProtInc
Negative regulation of transcription by RNA polymerase II Source: UniProtKB
Neuron apoptotic process Source: UniProtKB
Cellular Location
Nucleus; 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.
Involvement in disease
Dentatorubral-pallidoluysian atrophy (DRPLA): Autosomal dominant neurodegenerative disorder characterized by a loss of neurons in the dentate nucleus, rubrum, glogus pallidus and Luys'body. Clinical features are myoclonus epilepsy, dementia, and cerebellar ataxia. Onset of the disease occurs usually in the second decade of life and death in the fourth.
Congenital hypotonia, epilepsy, developmental delay, and digital anomalies (CHEDDA): An autosomal dominant neurodevelopmental syndrome characterized by severe global developmental delay, impaired intellectual development, poor or absent language, significant motor disability with inability to walk, dysmorphic facial features, skeletal anomalies, and variable congenital malformations. Most patients also have seizures and structural brain abnormalities.
PTM
Phosphorylated in vitro by MAPK8/JNK1 on Ser-739. Mutant ATN1 sequences with expanded poly-Gln (polyQ) traits are more slowly phosphorylated.
Proteolytically cleaved, probably in the nucleus, to produce two C-terminal fragments of 140 kDa (F1) and 125 kDa (F2) each containing poly-Gln (polyQ) tracts. F2 is produced by cleavage by caspases and is exported into the cytoplasm. In vitro, cleavage increases with an increase in the number of polyQ tracts. C-terminal proteolytic products appear to be the cause of cell toxicity. In vitro cleavage at Asp-109.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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