Product Name
Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (MCCC1), Recombinant Protein
Full Product Name
Recombinant Human Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (MCCC1)
Product Gene Name
MCCC1 recombinant protein
[Similar Products]
Product Synonym Gene Name
MCCC1[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence Positions
42-725aa, Full Length of Mature Protein
Sequence
TTATGRNITK VLIANRGEIA CRVMRTAKKL GVQTVAVYSE ADRNSMHVDM ADEAYSIGPA PSQQSYLSME KIIQVAKTSA AQAIHPGCGF LSENMEFAEL CKQEGIIFIG PPPSAIRDMG IKSTSKSIMA AAGVPVVEGY HGEDQSDQCL KEHARRIGYP VMIKAVRGGG GKGMRIVRSE QEFQEQLESA RREAKKSFND DAMLIEKFVD TPRHVEVQVF GDHHGNAVYL FERDCSVQRR HQKIIEEAPA PGIKSEVRKK LGEAAVRAAK AVNYVGAGTV EFIMDSKHNF CFMEMNTRLQ VEHPVTEMIT GTDLVEWQLR IAAGEKIPLS QEEITLQGHA FEARIYAEDP SNNFMPVAGP LVHLSTPRAD PSTRIETGVR QGDEVSVHYD PMIAKLVVWA ADRQAALTKL RYSLRQYNIV GLHTNIDFLL NLSGHPEFEA GNVHTDFIPQ HHKQLLLSRK AAAKESLCQA ALGLILKEKA MTDTFTLQAH DQFSPFSSSS GRRLNISYTR NMTLKDGKNN VAIAVTYNHD GSYSMQIEDK TFQVLGNLYS EGDCTYLKCS VNGVASKAKL IILENTIYLF SKEGSIEIDI PVPKYLSSVS SQETQGGPLA PMTGTIEKVF VKAGDKVKAG DSLMVMIAMK MEHTIKSPKD GTVKKVFYRE GAQANRHTPL VEFEEEESDK RESE
3D Structure
ModBase 3D Structure for Q96RQ3
Host
E Coli or Yeast or Baculovirus or Mammalian Cell
Purity/Purification
>85% (SDS-PAGE) (lot specific)
Form/Format
Liquid containing glycerol
Tag Information
This protein contains an N-terminal tag and may also contain a C-terminal tag. Tag types are determined by various factors including tag-protein stability, please inquire for tag information.
Sterility
Sterile filter available upon request.
Endotoxin
Low endotoxin available upon request.
Storage Buffer
Tris-based buffer, 50% glycerol
Preparation and Storage
Store at -20 degrees C. For long-term storage, store at -20 degrees C or -80 degrees C. Store working aliquots at 4 degrees C for up to one week. Repeated freezing and thawing is not recommended.
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of MCCC1 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
MCCC1 recombinant protein
This gene encodes the large subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism.
NCBI/Uniprot data below describe general gene information for MCCC1. It may not necessarily be applicable to this product.
NCBI Accession #
NP_001280202.1
[Other Products]
NCBI GenBank Nucleotide #
NM_001293273.1
[Other Products]
UniProt Primary Accession #
Q96RQ3
[Other Products]
UniProt Secondary Accession #
Q59ES4; Q9H959; Q9NS97[Other Products]
UniProt Related Accession #
Q96RQ3[Other Products]
Molecular Weight
80,473 Da
NCBI Official Full Name
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 2
NCBI Official Synonym Full Names
methylcrotonoyl-CoA carboxylase 1
NCBI Official Symbol
MCCC1??[Similar Products]
NCBI Official Synonym Symbols
MCCA; MCC-B
??[Similar Products]
NCBI Protein Information
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
UniProt Protein Name
Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
UniProt Synonym Protein Names
3-methylcrotonyl-CoA carboxylase 1; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha
UniProt Gene Name
MCCC1??[Similar Products]
UniProt Synonym Gene Names
MCCA; MCCase subunit alpha??[Similar Products]
NCBI Summary for MCCC1
This gene encodes the large subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism. [provided by RefSeq, Jul 2008]
UniProt Comments for MCCC1
Biotin-attachment subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism.
Research Articles on MCCC1
1. This study reports data mainly obtained from the Portuguese newborn screening program collected over a ten-year period. Analysis of the MCCC1 and MCCC2 genes yielded 26 previously unreported mutations and a variant of clinically unknown significance.
Precautions
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Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.
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