Twinkle/ATXN8 Antibody, ALEXA FLUOR 350
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Catalog number
bs-11775R-A350
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Price
Please ask
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Size
100ul
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Long name
Twinkle/ATXN8 Polyclonal Antibody, ALEXA FLUOR 350 Conjugated
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Also known as
Anti-Twinkle/ATXN8 PAb ALEXA FLUOR 350
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Category
Conjugated Primary Antibodies
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Conjugated with
ALEXA FLUOR® 350
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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
This antibody was obtained by immunization of the host with 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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Cross reactive species details
Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
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Background of the antigen
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
Purified by Protein A.
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Storage conditions
Store this antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Keep refrigerated at 2 to 8 degrees Celcius for up to one year.
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Excitation emission
343nm/442nm
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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
For facs or microscopy Alexa 1 conjugate. Alexa Fluor 350 conjugates can be used in multi-color flow cytometry with FACS's equipped with a second red laser or red diode. If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies. ALEXA FLUOR they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.
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Conjugation
Alexa Fluor, ALEXA FLUOR 350
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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
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Isotype
Immunoglobulin G (IgG)
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Label
ALEXA FLUOR 350
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Alternative name
Twinkle/ATXN8 Polyclonal Antibody, ALEXA FLUOR 350
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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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