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Twinkle/ATXN8 Antibody, Cy3 Conjugated

  • Long name
    Twinkle/ATXN8 Polyclonal Antibody, Cy3 Conjugated
  • Category
    Conjugated Primary Antibodies
  • Conjugation
    Cy3
  • Host Organism
    Rabbit (Oryctolagus cuniculus)
  • Target Antigen
    Twinkle/ATXN8
  • Specificity
    This is a highly specific antibody against Twinkle/ATXN8.
  • Modification
    Unmodified
  • Modification site
    None
  • Clonality
    Polyclonal
  • Clone
    Polyclonal antibody
  • Concentration
    1ug per 1ul
  • Source
    KLH conjugated synthetic peptide derived from human Twinkle/ATXN8
  • Tested applications
    IF(IHC-P)
  • Recommended dilutions
    IF(IHC-P)(1:50-200)
  • Crossreactivity
    Human, Mouse, Rat
  • Crossreactive species details
    Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
  • 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.
  • Purification method
    This antibody was purified via Protein A.
  • 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.
  • Excitation Emission
    512,550nm/570,615nm
  • 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.
  • 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 Twinkle/ATXN8 Antibody, Conjugated 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.
  • Additional conjugation
    cy3 conjugation kit
  • French translation
    anticorps
  • Gene target
  • Short name
    Twinkle/ATXN8 Antibody, Conjugated
  • Technique
    Antibody, antibodies against human proteins, antibodies for, antibody Conjugates
  • Label
    Cy3
  • Alternative name
    Twinkle/ATXN8 (Antibody to), cyanine 3 coupled
  • Alternative technique
    antibodies
1. Gene info
MeSH Data
  • Name
  • 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.
  • Tree numbers
    • E05.196.401.143
    • E05.301.300.096
    • E05.478.566.320.200
    • E05.601.262
    • E05.601.470.320.200
  • Qualifiers
    classification, economics, history, instrumentation, methods, standards, trends, utilization, veterinary, statistics & numerical data, ethics
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