Twinkle/ATXN8 Antibody, Cy3 Conjugated
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Catalog number
bs-11775R-Cy3
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Price
Please ask
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Size
0.1ml
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Long name
Twinkle/ATXN8 Polyclonal Antibody, Cy3 Conjugated
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Category
Conjugated Primary Antibodies
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Conjugation
Cy3
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Host Organism
Rabbit (Oryctolagus cuniculus)
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Target Antigen
Twinkle/ATXN8
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Specificity
This is a highly specific antibody against Twinkle/ATXN8.
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Modification
Unmodified
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Modification site
None
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Clonality
Polyclonal
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Clone
Polyclonal antibody
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Concentration
1ug per 1ul
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Source
KLH conjugated synthetic peptide derived from human Twinkle/ATXN8
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Tested applications
IF(IHC-P)
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Recommended dilutions
IF(IHC-P)(1:50-200)
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Crossreactivity
Human, Mouse, Rat
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Crossreactive species details
Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
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Antigen background
Twinkle, also known as PEO1 (Progressive external ophthalmoplegia 1 protein), PEOA3, SANDO or TWINL, is a mitochondrial protein that functions as a 5’-3’ nucleotide-dependent DNA helicase. Colocalized with mtDNA (mitochondrial DNA) in mitochondrial nucleoids, Twinkle is important in the metabolism and maintenance of mtDNA, playing a crucial role in the regulation of mtDNA copy numbers. Twinkle is expressed at high levels in testis, pancreas and skeletal muscle and exists as three isoforms due to alternative splicing events. Defects in the gene encoding Twinkle are the cause of two conditions: progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant 3 (PEOA3) and sensory ataxic neuropathy dysarthria and ophthalmoparesis (SANDO). PEOA3 is characterized by ptosis and weak muscles, while SANDO is characterized by ophthalmoparesis, dysarthria and sensory ataxic neuropathies.
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Purification method
This antibody was purified via Protein A.
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Storage conditions
Keep the antibody in an aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store refrigerated at 2 to 8 degrees Celcius for up to 1 year.
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Excitation Emission
512,550nm/570,615nm
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Synonyms
Ataxin 8;Ataxin8;ATXN 8;ATXN8;C10 orf2;C10orf 2;C10orf2;Chromosome 10 open reading frame 2;IOSCA;PEO 1;PEO;PEO1;PEOA3;Progressive external ophthalmoplegia 1 protein;SANDO;SCA 8;SCA8;T7 gp4 like protein with intramitochondrial nucleoid localization;T7 like mitochondrial DNA helicase;Twinkle protein, mitochondrial;TWINL.
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Properties
If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies they should be stored frozen at - 24°C for long term storage and for short term at + 5°C. Cy3 antibodies are excited by the 488-nanometer wave of an argon laser and the 633-nanometer line of a helium-neon diode laser. 1 of the Anti-Twinkle/ATXN8 can be used in flow cytometry but typically shows lower fluorescence intensity comparable to that of PE or APC. This Bioss Primary Conjugated Antibodies antibody is well suited for fluorescent microscopy.
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Additional conjugation
cy3 conjugation kit
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French translation
anticorps
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Gene target
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Gene symbol
ATXN8
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Short name
Anti-Twinkle/ATXN8
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Technique
Antibody, antibodies against human proteins, antibodies for, antibody Conjugates
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Isotype
Immunoglobulin G (IgG)
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Label
Cy3
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Alternative name
Anti-Twinkle/ATXN8 PAb Cy3
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Alternative technique
antibodies
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Gene info
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Identity
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Gene
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Long gene name
ataxin 8
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GenBank acession
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Locus
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Discovery year
2006-07-18
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Entrez gene record
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Pubmed identfication
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Classification
MeSH Data
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Name
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Concept
Scope note:
Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.
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Tree numbers
- E05.196.401.143
- E05.301.300.096
- E05.478.566.320.200
- E05.601.262
- E05.601.470.320.200
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Qualifiers
ethics, trends, veterinary, history, classification, economics, instrumentation, methods, standards, statistics & numerical data
Product images
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