Analytical Data
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Gene name
GCDH
- Application
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Alternative Names
GCDH;Glutaryl-CoA dehydrogenase. 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
Q92947
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Expression Region
45-438aa
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AA Sequence
MGSSHHHHHHSSGLVPRGSHMRPEFDWQDPLVLEEQLTTDEILIRDTFRT YCQERLMPRILLANRNEVFHREIISEMGELGVLGPTIKGYGCAGVSSVAY GLLARELERVDSGYRSAMSVQSSLVMHPIYAYGSEEQRQKYLPQLAKGEL LGCFGLTEPNSGSDPSSMETRAHYNSSNKSYTLNGTKTWITNSPMADLFV VWARCEDGCIRGFLLEKGMRGLSAPRIQGKFSLRASATGMIIMDGVEVPE ENVLPGASSLGGPFGCLNNARYGIAWGVLGASEFCLHTARQYALDRMQFG VPLARNQLIQKKLADMLTEITLGLHACLQLGRLKDQDKAAPEMVSLLKRN NCGKALDIARQARDMLGGNGISDEYHVIRHAMNLEAVNTYEGTHDIHALI LGRAITGIQAFTASK
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Molecular Weight
46 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
GCDH (Glutaryl-CoA Dehydrogenase) is a crucial enzyme involved in the catabolism of lysine, hydroxylysine, and tryptophan, playing a significant role in the metabolic pathway that converts these amino acids into acetoacetate and acetyl-CoA. Mutations in the GCDH gene lead to glutaryl-CoA dehydrogenase deficiency (GCDH deficiency), a rare but severe metabolic disorder characterized by the accumulation of toxic metabolites, resulting in neurological impairment, movement disorders, and metabolic crises. The incidence of GCDH deficiency varies globally, with a particularly high prevalence in certain populations, emphasizing the need for effective diagnostic and therapeutic strategies. Research on GCDH recombinant proteins has gained traction as it provides insights into enzyme function, structure, and potential therapies. Producing GCDH as a recombinant protein allows for better understanding of its catalytic mechanisms, substrate interactions, and the impact of specific mutations associated with the deficiency. Furthermore, recombinant GCDH has the potential for therapeutic applications, including enzyme replacement therapy and development of small molecules to restore enzyme activity in affected individuals. The advancement of protein engineering techniques and high-throughput screening methods presents new avenues for developing effective treatments, enhancing our understanding of the disease, and addressing the metabolic imbalance in patients. Thus, GCDH recombinant protein research stands at the intersection of fundamental biochemistry and clinical application, representing a hopeful frontier in the fight against inherited metabolic disorders.











