Product Name
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCC2), Recombinant Protein
Full Product Name
Recombinant Human Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCC2)
Product Gene Name
MCCC2 recombinant protein
[Similar Products]
Product Synonym Gene Name
MCCC2[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence Positions
23-563aa, Full Length of Mature Protein
Sequence
YHGDSVASLG TQPDLGSALY QENYKQMKAL VNQLHERVEH IKLGGGEKAR ALHISRGKLL PRERIDNLID PGSPFLELSQ FAGYQLYDNE EVPGGGIITG IGRVSGVECM IIANDATVKG GAYYPVTVKK QLRAQEIAMQ NRLPCIYLVD SGGAYLPRQA DVFPDRDHFG RTFYNQAIMS SKNIAQIAVV MGSCTAGGAY VPAMADENII VRKQGTIFLA GPPLVKAATG EEVSAEDLGG ADLHCRKSGV SDHWALDDHH ALHLTRKVVR NLNYQKKLDV TIEPSEEPLF PADELYGIVG ANLKRSFDVR EVIARIVDGS RFTEFKAFYG DTLVTGFARI FGYPVGIVGN NGVLFSESAK KGTHFVQLCC QRNIPLLFLQ NITGFMVGRE YEAEGIAKDG AKMVAAVACA QVPKITLIIG GSYGAGNYGM CGRAYSPRFL YIWPNARISV MGGEQAANVL ATITKDQRAR EGKQFSSADE AALKEPIIKK FEEEGNPYYS SARVWDDGII DPADTRLVLG LSFSAALNAP IEKTDFGIFR M
3D Structure
ModBase 3D Structure for Q9HCC0
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 MCCC2 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
MCCC2 recombinant protein
This gene encodes the small 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 MCCC2. It may not necessarily be applicable to this product.
NCBI Accession #
NP_071415.1
[Other Products]
NCBI GenBank Nucleotide #
NM_022132.4
[Other Products]
UniProt Primary Accession #
Q9HCC0
[Other Products]
UniProt Secondary Accession #
Q96C27; Q9Y4L7; A6NIY9[Other Products]
UniProt Related Accession #
Q9HCC0[Other Products]
Molecular Weight
57,519 Da
NCBI Official Full Name
methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
NCBI Official Synonym Full Names
methylcrotonoyl-CoA carboxylase 2
NCBI Official Symbol
MCCC2 [Similar Products]
NCBI Official Synonym Symbols
MCCB
[Similar Products]
NCBI Protein Information
methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
UniProt Protein Name
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
UniProt Synonym Protein Names
3-methylcrotonyl-CoA carboxylase 2; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta
Protein Family
Methylcrotonoyl-CoA carboxylase
UniProt Gene Name
MCCC2 [Similar Products]
UniProt Synonym Gene Names
MCCB; MCCase subunit beta [Similar Products]
NCBI Summary for MCCC2
This gene encodes the small 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 MCCC2
Carboxyltransferase 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 MCCC2
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
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
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.
It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
