Analytical Data
-
Gene name
MCCC1
- Application
-
Alternative Names
MCCC1；SIR2L4；NAD-dependent Protein lipoamidase sirtuin-4. mitochondrial
-
Species
Human
-
Source
E. coli
-
Tag
His tag N-Terminus
-
Purity
Greater than 90% as determined by SDS-PAGE.
-
Uniprot
Q96RQ3
-
Expression Region
1-725aa
-
AA Sequence
MAAASAVSVLLVAAERNRWHRLPSLLLPPRTWVWRQRTMKYTTATGRNIT KVLIANRGEIACRVMRTAKKLGVQTVAVYSEADRNSMHVDMADEAYSIGP APSQQSYLSMEKIIQVAKTSAAQAIHPGCGFLSENMEFAELCKQEGIIFI GPPPSAIRDMGIKSTSKSIMAAAGVPVVEGYHGEDQSDQCLKEHARRIGY PVMIKAVRGGGGKGMRIVRSEQEFQEQLESARREAKKSFNDDAMLIEKFV DTPRHVEVQVFGDHHGNAVYLFERDCSVQRRHQKIIEEAPAPGIKSEVRK KLGEAAVRAAKAVNYVGAGTVEFIMDSKHNFCFMEMNTRLQVEHPVTEMI TGTDLVEWQLRIAAGEKIPLSQEEITLQGHAFEARIYAEDPSNNFMPVAG PLVHLSTPRADPSTRIETGVRQGDEVSVHYDPMIAKLVVWAADRQAALTK LRYSLRQYNIVGLPTNIDFLLNLSGHPEFEAGNVHTDFIPQHHKQLLLSR KAAAKESLCQAALGLILKEKAMTDTFTLQAHDQFSPFSSSSGRRLNISYT RNMTLKDGKNNVAIAVTYNHDGSYSMQIEDKTFQVLGNLYSEGDCTYLKC SVNGVASKAKLIILENTIYLFSKEGSIEIDIPVPKYLSSVSSQETQGGPL APMTGTIEKVFVKAGDKVKAGDSLMVMIAMKMEHTIKSPKDGTVKKVFYR EGAQANRHTPLVEFEEEESDKRESE
-
Molecular Weight
80.4 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
MCCC1, or methylcrotonyl-CoA carboxylase 1, plays a crucial role in the catabolism of amino acids, particularly leucine, by facilitating the conversion of methylcrotonyl-CoA to 3-methylglutaconyl-CoA in the mitochondria. Mutations in the MCCC1 gene are associated with a rare metabolic disorder known as methylcrotonyl-CoA carboxylase deficiency, which can lead to various symptoms, including developmental delays, metabolic crises, and neurological complications. The study of MCCC1 recombinant protein has gained significant attention due to its potential implications for understanding the biochemical pathways involved in this disorder and developing therapeutic strategies. By producing recombinant MCCC1, researchers aim to elucidate its structure, function, and enzymatic properties, providing insights into how mutations affect its activity. Additionally, understanding the molecular mechanisms underpinning MCCC1-related disorders can pave the way for novel diagnostic tools and targeted gene therapies. Through these studies, the hope is to improve patient outcomes and deepen our understanding of human metabolic processes.
