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Twinkle/ATXN8 Antibody, Cy3 Conjugated

  • Catalog number
    bs-11775R-Cy3
  • Product name
    Twinkle/ATXN8 Antibody, Cy3 Conjugated
  • Size
    0.1ml
  • Price
    350EUR
  • Supplier
    Bioss Primary Conjugated Antibodies
  • Ask
  • Clonality
    Polyclonal
  • Host Organism
    Rabbit (Oryctolagus cuniculus)
  • 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.
  • Modification site
    None
  • Crossreactive species details
    Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
  • Long name
    Twinkle/ATXN8 Polyclonal Antibody, Cy3 Conjugated
  • 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.
  • Recommended dilutions
    IF(IHC-P)(1:50-200)
  • 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.
  • Conjugation
    Cy3
  • Modification
    Unmodified
  • Category
    Conjugated Primary Antibodies
  • Source
    KLH conjugated synthetic peptide derived from human Twinkle/ATXN8
  • Specificity
    This is a highly specific antibody against Twinkle/ATXN8.
  • Tested applications
    IF(IHC-P)
  • Clone
    Polyclonal antibody
  • Crossreactivity
    Human, Mouse, Rat
  • Concentration
    1ug per 1ul
  • Target Antigen
    Twinkle/ATXN8
  • Excitation Emission
    512,550nm/570,615nm
  • 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
  • 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
  • Ask
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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