| Long name: |
Biotin Conjugated, Twinkle/ATXN8 Polyclonal Antibody |
| Category: |
Conjugated Primary Antibodies |
| Conjugation: |
Biotinylated |
| 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: |
IHC-P, WB |
| Recommended dilutions: |
IHC-P(1:100-500), WB(1:100-1000) |
| Crossreactivity: |
Mouse, Rat, Human |
| Crossreactive species details: |
not every possible cross-reactivity is known, Due to limited amount of testing and knowledge |
| Antigen background: |
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 refrigerated at 2 to 8 degrees Celcius for up to 1 year, 09% sodium azide, Keep the antibody in an aqueous buffered solution containing 1% BSA |
| Properties: |
Avidin and Streptavidin bind to the small biotin and are couple to HRP or AP for ELISA, Biotin conjugates can be detected by horseradish peroxidase, To break the streptavidin Biotin bond we suggest to use a 6 molar guanidine HCl solution with acidity of pH 1, alkaline phosphatase substrates or anti biotin conjugated antibodies, 6, C, C for long term storage and for short term at + 5°, If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies they should be stored frozen at - 24° |
| Gene target: |
Twinkle/ATXN8 Conjugated |
| Short name: |
Conjugated, Twinkle/ATXN8 Antibody |
| Technique: |
antibodies against human proteins, antibodies for, antibody Conjugates, Antibody |
| Label: |
Biotin |
| Alternative name: |
biotinilated coupled, 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 |