Description:

Size: 100ul

Catalog no.: bs-11775R-A647

Price: 380 EUR

Product details

Modification Site

None

French translation

anticorps

Tested applications

IF(IHC-P)

Clonality

Polyclonal

Modification

Unmodified

Concentration

1ug per 1ul

Excitation emission

650nm/665nm

Target Antigen

Twinkle/ATXN8

Conjugated with

ALEXA FLUOR® 647

Crossreactivity

Human, Mouse, Rat

Recommended dilutions

IF(IHC-P)(1:50-200)

Clone

Polyclonal antibody

Purification

Purified by Protein A.

Conjugation

Alexa Fluor,ALEXA FLUOR 647

Category

Conjugated Primary Antibodies

Host Organism

Rabbit (Oryctolagus cuniculus)

Also known as

Anti-Twinkle/ATXN8 PAb ALEXA FLUOR 647

Specificity

This is a highly specific antibody against Twinkle/ATXN8.

Long name

Twinkle/ATXN8 Polyclonal Antibody, ALEXA FLUOR 647 Conjugated

Cross-reactive species details

Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.

Source

This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human Twinkle/ATXN8

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.

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

For facs or microscopy Alexa 1 conjugate.Alexa Fluor 633 is a practical alternative to APC as well as Cy5. Bioss Primary Conjugated Antibodies. ALEXA FLUOR made this Alexa Fluor 633 conjugate that can be used in multi-color flow cytometry with instruments equipped with a second red laser or red diode. It is detected in the FL4 detector of the core's upgraded 2-laser FACScans. Like other Alexa Fluor dyes, the Anti-Twinkle/ATXN8 exhibits uncommon photo stability, making it an ideal choice for fluorescent microscopy.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.

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.