Analytical Data
-
Gene name
ATXN8
- Application
-
Alternative Names
Ataxin 8; Ataxin8; ATXN 8; ATXN8; C10 orf2 ; C10orf 2 ; C10orf2; Chromosome 10 open reading frame 2; IOSCA; mitochondrial; MTDPS7; PEO 1; PEO; PEO1; PEO1_HUMAN; PEOA3; Progressive external ophthalmoplegia 1 protein; SANDO; SCA 8; SCA8; T7 gp4 like protein with intramitochondrial nucleoid localization
-
Species
Human
-
Source
E. coli
-
Tag
His tag N-Terminus
-
Purity
Greater than 90% as determined by SDS-PAGE.
-
Uniprot
Q96RR1
-
Expression Region
32-684aa
-
AA Sequence
APGPPRRRYRKETLQALDMPVLPVTATEIRQYLRGHGIPFQDGHSCLRALSPFAESSQLKGQTGVTTSFSLFIDKTTGHFLCMTSLAEGSWEDFQASVEGRGDGAREGFLLSKAPEFEDSEEVRRIWNRAIPLWELPDQEEVQLADTMFGLTKVTDDTLKRFSVRYLRPARSLVFPWFSPGGSGLRGLKLLEAKCQGDGVSYEETTIPRPSAYHNLFGLPLISRRDAEVVLTSRELDSLALNQSTGLPTLTLPRGTTCLPPALLPYLEQFRRIVFWLGDDLRSWEAAKLFARKLNPKRCFLVRPGDQQPRPLEALNGGFNLSRILRTALPAWHKSIVSFRQLREEVLGELSNVEQAAGLRWSRFPDLNRILKGHRKGELTVFTGPTGSGKTTFISEYALDLCSQGVNTLWGSFEISNVRLARVMLTQFAEGRLEDQLDKYDHWADRFEDLPLYFMTFHGQQSIRTVIDTMQHAVYVYDICHVIIDNLQFMMGHEQLSTDRIAAQDYIIGVFRKFATDNNCHVTLVIHPRKEDDDKELQTASIFGSAKASQEADNVLILQDRKLVTGPGKRYLQVSKNRFDGDVGVFPLEFNKNSLTFSIPPKNKARLKKIKDDTGPVAKKPSSGKKGATTQNSEICSGQAPTPDQPDTSKRSK
-
Molecular Weight
75.5 kDa
-
Endotoxin
< 1.0 EU per μg protein as determined by the LAL method.
-
Form
Freeze-dried powder
-
Buffer formulation
PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
-
Reconstitution
Reconstitute in ddH2O to a concentration of 0.1-0.5 mg/mL. Do not vortex.
- Customization
-
Stability Test
The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37℃ for 48h, and no obvious degradation and precipitation were observed. The loss rate isless than 8% within the expiration date under appropriate storage condition.
-
Storage & Shelf Life
Samples are stable for up to twelve months from date of receipt at -20℃ to -80℃. Store it under sterile conditions at -20℃ to -80℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
-
Shipping
In general, recombinant proteins are supplied as lyophilized powder and shipped at ambient temperature. For bulk packages, the proteins are provided as frozen liquid and shipped with blue ice, unless otherwise requested by the customer.
Quality inspection process
Related Products
Protein Description
ATXN8 is a gene associated with the neurodegenerative disorder Spinocerebellar Ataxia Type 8 (SCA8), which is characterized by progressive coordination and balance difficulties due to the degeneration of neuronal structures. The research surrounding ATXN8 recombinant proteins has gained significant attention in recent years, driven by the need to understand the underlying mechanisms of SCA8 and to explore potential therapeutic avenues. ATXN8 encodes an expansion of CAG repeats, which can lead to the production of toxic polyglutamine (polyQ) proteins. These proteins are believed to misfold and aggregate, contributing to neuronal toxicity. By studying ATXN8 recombinant proteins, researchers aim to elucidate the molecular pathways involved in SCA8 pathogenesis, identify critical interactions with cellular components, and assess how these interactions may influence disease progression. Furthermore, the generation of recombinant ATXN8 proteins facilitates high-throughput screening of pharmacological agents that may mitigate toxic effects or enhance cellular resilience. Understanding the conformation, aggregation propensity, and cellular effects of these recombinant proteins not only provides insights into SCA8 but also holds promise for broader applications in treating other polyglutamine disorders. As research progresses, recombinant ATXN8 proteins may become crucial tools for developing diagnostic markers and therapeutic strategies, offering hope for individuals affected by SCA8 and similar conditions.
