Analytical Data
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Gene name
C9orf72
- Application
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Alternative Names
ALSFTD; C9orf72; chromosome 9 open reading frame 72; CI072_HUMAN; FTDALS; MGC23980; Protein C9orf72; RP11-27J8.2; Uncharacterized Protein C9orf72
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Species
Human
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Source
E. coli
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Tag
GST-tag at N-terminal
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Purity
Greater than 90% as determined by SDS-PAGE.
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Uniprot
Q96LT7
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Expression Region
1-481aa
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AA Sequence
MSTLCPPPSP AVAKTEIALS GKSPLLAATF AYWDNILGPR VRHIWAPKTE QVLLSDGEIT FLANHTLNGE ILRNAESGAI DVKFFVLSEK GVIIVSLIFD GNWNGDRSTY GLSIILPQTE LSFYLPLHRV CVDRLTHIIR KGRIWMHKER QENVQKIILE GTERMEDQGQ SIIPMLTGEV IPVMELLSSM KSHSVPEEID IADTVLNDDD IGDSCHEGFL LNAISSHLQT CGCSVVVGSS AEKVNKIVRT LCLFLTPAER KCSRLCEAES SFKYESGLFV QGLLKDSTGS FVLPFRQVMY APYPTTHIDV DVNTVKQMPP CHEHIYNQRR YMRSELTAFW RATSEEDMAQ DTIIYTDESF TPDLNIFQDV LHRDTLVKAF LDQVFQLKPG LSLRSTFLAQ FLLVLHRKAL TLIKYIEDDT QKGKKPFKSL RNLKIDLDLT AEGDLNIIMA LAEKIKPGLH SFIFGRPFYT SVQERDVLMT F
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Molecular Weight
54.3 KDa
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Endotoxin
< 1.0 EU per μg protein as determined by the LAL method.
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Form
Freeze-dried powder
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Buffer formulation
PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
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Reconstitution
Reconstitute in ddH2O to a concentration of 0.1-0.5 mg/mL. Do not vortex.
- Customization
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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.
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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.
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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
C9orf72, located on chromosome 9, is the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). The gene is primarily known for a hexanucleotide repeat expansion (GGGGCC) within its intron, which leads to the production of aberrant dipeptide repeat proteins through a process called repeat-associated non-AUG (RAN) translation. These dipeptide repeat proteins accumulate in brain and spinal cord tissues, contributing to neurodegenerative processes. Research has indicated that the presence of these aggregates is associated with cytotoxicity, inflammation, and impaired cellular functions, creating a complex relationship between these proteins and the associated pathologies. Understanding the mechanisms by which C9orf72-derived proteins induce neurodegeneration is crucial for developing targeted therapies. Furthermore, studies have explored the role of RNA foci formed by the expanded repeat sequences and their toxic effects on cellular homes, leading to insights into potential interventions. Investigating C9orf72's biological functions and its impact on cellular pathways is essential for uncovering new treatment strategies for ALS and FTD, making it a significant focus in neurodegenerative disease research. Overall, the study of C9orf72 and its derived proteins bridges genetics, molecular biology, and neurobiology, emphasizing the need for a comprehensive approach to tackle these devastating disorders.
