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ATXN3 Polyclonal Antibody

CAT: 0763-E-AB-52456-01Size: 20 µLDry Ice: NoHazardous: No
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CAT#:0763-E-AB-52456-01Size:20 µL
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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Background
Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by this gene contains (CAG) n repeats in the coding region, and the expansion of these repeats from the normal 12-44 to 52-86 is one cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. Alternatively spliced transcript variants encoding different isoforms have been described for this gene.
Abbreviation
ATXN3
UniProt
P54252
Host
Rabbit
Reactivity
Human
Immunogen
Fusion protein of human ATXN3
Target
AT3; Ataxin 3; ataxin 3 variant h; ataxin 3 variant m; ataxin 3 variant ref; Ataxin-3; ATX3; ATX3; ATXN3; EC 3.4.22.; JOS; Josephin; Machado Joseph disease (spinocerebellar ataxia 3; olivopontocerebellar ataxia 3; autosomal dominant; ataxin 3) ; Machado Joseph disease; Machado Joseph disease protein 1; Machado-Joseph disease protein 1; Machado-Joseph disease protein 1 homolog; MJD; MJD gene; MJD1; Olivopontocerebellar ataxia 3; OTTHUMP00000221583; OTTHUMP00000221585; OTTHUMP00000221586; OTTHUMP00000221587; OTTHUMP00000231995; OTTHUMP00000231997; Rsca3; SCA3; SCA3 gene; Spinocerebellar ataxia type 3 protein
Clonality
Polyclonal
Conjugation
Unconjugated
Applications
IHC
Field of Research
Cancer; Epigenetics and Nuclear Signaling; Neuroscience
Purification
Antigen affinity purification
Concentration
0.4 mg/mL
Dilution
IHC 1:40-1:200
Buffer
PBS with 0.05% NaN3 and 40% Glycerol, pH7.4
Shipping Conditions
The product is shipped with ice pack, upon receipt, store it immediately at the temperature recommended.
Storage Conditions
Store at -20°C Valid for 12 months. Avoid freeze / thaw cycles.
Isotype
IgG

UniProtKB · P54252

Ataxin-3

ATX3_HUMAN · Homo sapiens

View on UniProt ↗
Primary accession
P54252
Review status
UniProtKB reviewed (Swiss-Prot)
Gene
ATXN3
Protein existence
1: Evidence at protein level
Organism
Homo sapiens (Human)
Taxonomy ID
9606
Alternative names
—
EC number
3.4.19.12
Processing
—
Secondary accessions
A7LFZ5, D6RDL9, E9PB63, O15284, O15285, O15286, Q8N189, Q96TC3, Q96TC4, Q9H3N0
Protein keywords

Technical term

3D-structureProteomics identificationReference proteome

Coding sequence diversity

Alternative splicingTriplet repeat expansion

Molecular function

HydrolaseProteaseThiol protease

PTM

Isopeptide bondPhosphoproteinUbl conjugation

Cellular component

LysosomeMembraneNucleus

Disease

NeurodegenerationSpinocerebellar ataxia

Domain

Repeat

Biological process

TranscriptionTranscription regulationUbl conjugation pathway

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