| Long name: |
Twinkle/ATXN8 Primary Polyclonal Antibody |
| Also known as: |
Twinkle/ATXN8 PAb |
| Category: |
Primary Antibodies |
| Conjugation: |
Unconjugated |
| Target Antigen: |
Twinkle/ATXN8 |
| Specificity: |
This is a highly specific antibody against Twinkle/ATXN8 |
| Modification(s): |
None |
| Modification site(s): |
Unmodified antibody |
| Clonality: |
Polyclonal antibody |
| Clone number: |
Polyclonal antibody |
| Concentration: |
1ug per 1ul |
| Source: |
This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human Twinkle/ATXN8 |
| Tested Applications: |
IF(IHC-P), IHC-P, WB |
| Recommended dilutions: |
IF(IHC-P)(1:50-200), IHC-P(1:100-500), WB(1:100-1000) |
| Cross reactivity: |
Mouse, Rat, Human |
| Cross reactive species details: |
not every possible cross-reactivity is known, Due to limited amount of testing and knowledge |
| Background of the target antigen: |
Colocalized with mtDNA (mitochondrial DNA) in mitochondrial nucleoids, 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, PEOA3 is characterized by ptosis and weak muscles, SANDO or TWINL, Twinkle is expressed at high levels in testis, Twinkle is important in the metabolism and maintenance of mtDNA, also known as PEO1 (Progressive external ophthalmoplegia 1 protein), dysarthria and sensory ataxic neuropathies, is a mitochondrial protein that functions as a 5â, nucleotide-dependent DNA helicase, pancreas and skeletal muscle and exists as three isoforms due to alternative splicing events, playing a crucial role in the regulation of mtDNA copy numbers, while SANDO is characterized by ophthalmoparesis, &euro, &euro, &trade, &trade, -3â, Twinkle |
| Purification method: |
This antibody was purified via Protein A |
| Storage conditions: |
50% glycerol and 0, Store at -20°, 09% sodium azide, C for up to 1 year, Keep the antibody in aqueous buffered solution containing 1% BSA |
| Synonym names: |
mitochondrial, ATXN 8, ATXN8, Ataxin 8, Ataxin8, C10 orf2, C10orf 2, C10orf2, Chromosome 10 open reading frame 2, IOSCA, PEO, PEO 1, PEO1, PEOA3, Progressive external ophthalmoplegia 1 protein, SANDO, SCA 8, SCA8, T7 gp4 like protein with intramitochondrial nucleoid localization, T7 like mitochondrial DNA helicase, TWINL, Twinkle protein |
| Properties: |
C, C for long term storage and for short term at + 5°, If you buy Antibodies supplied by Bioss Primary Unconjugated Antibodies they should be stored frozen at - 24° |
| Gene target: |
Twinkle/ATXN8 |
| Short name: |
Twinkle/ATXN8 Antibody |
| Technique: |
antibodies against human proteins, antibodies for, Antibody |
| Alternative name: |
Twinkle/ATXN8 (Antibody to) |
| Alternative technique: |
antibodies |
| Identity: |
32925 |
| Gene: |
ATXN8 |
More about : ATXN8 |
| Long gene name: |
ataxin 8 |
| Locus: |
13q21, 33 |
| Discovery year: |
2006-07-18 |
| GenBank acession: |
DQ641254 |
| Entrez gene record: |
724066 |
| Pubmed identfication: |
16804541 |
| Classification: |
Ataxins |