Twinkle/ATXN8 Polyclonal Antibody, Biotin Conjugated

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Cat# bs-11775R-Biotin

Size : 100ul

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bs-11775R-Biotin [Conjugated Primary Antibody]

Twinkle/ATXN8 Polyclonal Antibody, Biotin Conjugated

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Host: Rabbit

Target Protein: Twinkle/ATXN8

Immunogen Range: 551-650/684

Clonality: Polyclonal

Isotype: IgG

Source: KLH conjugated synthetic peptide derived from human Twinkle/ATXN8

Purification: Purified by Protein A.

Storage Buffer: Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Store at -20°C for 12 months.

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.

Conjugation: Biotin

Excitation/ Emission: N/A

Size: 100ul

Concentration: 1ug/ul

Applications: WB(1:300-5000)
ELISA(1:500-1000)

Predicted Molecular Weight: 77

Predicted Cross Reactive Species: Human
Mouse
Rat
Dog
Cow
Pig
Horse
Chicken
Rabbit

For research use only. Not intended for diagnostic or therapeutic use.

PRODUCT SPECIFIC PUBLICATIONS

owen Huet al. Mutation of TWNK Gene Is One of the Reasons of Runting and Stunting Syndrome Characterized by mtDNA Depletion in Sex-Linked Dwarf Chicken. Front Cell Dev Biol. 2020 Jul 14;8:581.Read more>>