Analytical Data
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Gene name
HADHB
- Application
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Alternative Names
HADHB;Trifunctional enzyme subunit beta. mitochondrial
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Species
Human
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Source
E. coli
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Tag
His tag N-Terminus
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Purity
Greater than 90% as determined by SDS-PAGE.
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Uniprot
P55084
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Expression Region
34-474aa
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AA Sequence
MGSSHHHHHH SSGLVPRGSH MGSAAPAVQTKTKKTLAKPNIRNVVVVDGVRTPFLLSGTSYKDLMPHDLA RAALTGLLHRTSVPKEVVDYIIFGTVIQEVKTSNVAREAALGAGFSDKTP AHTVTMACISANQAMTTGVGLIASGQCDVIVAGGVELMSDVPIRHSRKMR KLMLDLNKAKSMGQRLSLISKFRFNFLAPELPAVSEFSTSETMGHSADRL AAAFAVSRLEQDEYALRSHSLAKKAQDEGLLSDVVPFKVPGKDTVTKDNG IRPSSLEQMAKLKPAFIKPYGTVTAANSSFLTDGASAMLIMAEEKALAMG YKPKAYLRDFMYVSQDPKDQLLLGPTYATPKVLEKAGLTMNDIDAFEFHE AFSGQILANFKAMDSDWFAENYMGRKTKVGLPPLEKFNNWGGSLSLGHPF GATGCRLVMAAANRLRKEGGQYGLVAACAAGGQGHAMIVEAYPK
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Molecular Weight
50 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
HADHB (hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta) is a crucial enzyme involved in the mitochondrial fatty acid oxidation pathway, specifically in the catabolism of long-chain fatty acids. Mutations in the HADHB gene lead to a rare but severe metabolic disorder known as mitochondrial trifunctional protein deficiency, characterized by hypoketotic hypoglycemia, muscle weakness, and increased accumulation of toxic fatty acid metabolites. The significance of HADHB extends beyond basic biochemistry; it has implications for understanding metabolic syndromes and developing potential therapeutic strategies. Researchers aim to elucidate the structural and functional characteristics of HADHB through recombinant protein techniques, which involve producing the protein in a host system, often Escherichia coli or yeast. Understanding the enzyme's activity, kinetics, and interactions with other metabolic pathways can provide insights into its role in cellular energy homeostasis and potential links to more common conditions such as obesity and type 2 diabetes. Moreover, recombinant HADHB may serve as a valuable tool for drug discovery and the assessment of therapeutic compounds aimed at correcting the enzyme's deficiencies. As such, studies of HADHB recombinant protein are integral not only to the basic understanding of mitochondrial function and energy metabolism but also to the broader landscape of metabolic disease research.
