Analytical Data
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Gene name
BCS1L
- Application
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Alternative Names
BCS1L; BCS1; Mitochondrial chaperone BCS1; h-BCS1; BCS1-like Protein
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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
Q9Y276
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Expression Region
1-419aa
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AA Sequence
MPLSDFILALKDNPYFGAGFGLVGVGTALALARKGVQLGLVAFRRHYMITLEVPARDRSYAWLLSWLTRHSTRTQHLSVETSYLQHESGRISTKFEFVPSPGNHFIWYRGKWIRVERSREMQMIDLQTGTPWESVTFTALGTDRKVFFNILEEARELALQQEEGKTVMYTAVGSEWRPFGYPRRRRPLNSVVLQQGLADRIVRDVQEFIDNPKWYTDRGIPYRRGYLLYGPPGCGKSSFITALAGELEHSICLLSLTDSSLSDDRLNHLLSVAPQQSLVLLEDVDAAFLSRDLAVENPVKYQGLGRLTFSGLLNALDGVASTEARIVFMTTNHVDRLDPALIRPGRVDLKEYVGYCSHWQLTQMFQRFYPGQAPSLAENFAEHVLRATNQISPAQVQGYFMLYKNDPVGAIHNAESLRR
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Molecular Weight
73.9 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
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Protein Description
BCS1L (BCS1-like) is a crucial mitochondrial protein that plays a significant role in the assembly of the mitochondrial respiratory complex V, also known as ATP synthase. Mutations in the BCS1L gene are associated with a range of mitochondrial diseases, particularly affecting energy metabolism and leading to symptoms such as encephalopathy, exercise intolerance, and developmental delays. The importance of BCS1L lies in its function in facilitating the insertion of specific subunits into the ATP synthase complex, thereby playing a key role in maintaining mitochondrial function and cellular energy production. Research into BCS1L has gained momentum as scientists aim to uncover the molecular mechanisms underlying its function, the consequences of genetic mutations, and potential therapeutic strategies for mitochondrial disorders. Functional studies of recombinant BCS1L proteins have provided insights into the protein’s structure, interaction with other mitochondrial components, and the impact of pathogenic variants. Understanding BCS1L's role in mitochondrial biogenesis and function is not only vital for elucidating the pathophysiology of related diseases but also holds promise for developing targeted therapies that could potentially alleviate symptoms or improve mitochondrial function in affected individuals. Overall, the study of BCS1L recombinant proteins is essential for advancing our knowledge of mitochondrial biology and for addressing the challenges associated with mitochondrial diseases.
