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BCKDHB recombinant protein :: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial (BCKDHB) Recombinant Protein

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Catalog # MBS960089
Unit / Price
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  0.05 mg (E-Coli)  /  $750 +1 FREE 8GB USB
  0.05 mg (Yeast)  /  $850 +1 FREE 8GB USB
  0.2 mg (E-Coli)  /  $1,005 +1 FREE 8GB USB
  0.05 mg (Baculovirus)  /  $1,080 +1 FREE 8GB USB
  0.5 mg (E-Coli)  /  $1,105 +1 FREE 8GB USB
  0.2 mg (Yeast)  /  $1,150 +1 FREE 8GB USB
  0.5 mg (Yeast)  /  $1,300 +1 FREE 8GB USB
  0.05 mg (Mammalian-Cell)  /  $1,320 +1 FREE 8GB USB
  0.1 mg (Baculovirus)  /  $1,560 +1 FREE 8GB USB
  1 mg (E-Coli)  /  $1,675 +2 FREE 8GB USB
  0.5 mg (Baculovirus)  /  $2,035 +2 FREE 8GB USB
  1 mg (Yeast)  /  $2,065 +2 FREE 8GB USB
  0.1 mg (Mammalian-Cell)  /  $2,145 +2 FREE 8GB USB
  1 mg (Baculovirus)  /  $3,145 +3 FREE 8GB USB
BCKDHB recombinant protein
Product Name

2-oxoisovalerate dehydrogenase subunit beta, mitochondrial (BCKDHB), Recombinant Protein

Full Product Name

Recombinant Human 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial (BCKDHB)

Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence Positions
51-392aa, Full Length of Mature Protein
Sequence
VAHFTFQPDP EPREYGQTQK MNLFQSVTSA LDNSLAKDPT AVIFGEDVAF GGVFRCTVGL RDKYGKDRVF NTPLCEQGIV GFGIGIAVTG ATAIAEIQFA DYIFPAFDQI VNEAAKYRYR SGDLFNCGSL TIRSPWGCVG HGALYHSQSP EAFFAHCPGI KVVIPRSPFQ AKGLLLSCIE DKNPCIFFEP KILYRAAAEE VPIEPYNIPL SQAEVIQEGS DVTLVAWGTQ VHVIREVASM AKEKLGVSCE VIDLRTIIPW DVDTICKSVI KTGRLLISHE APLTGGFASE ISSTVQEECF LNLEAPISRV CGYDTPFPHI FEPFYIPDKW KCYDALRKMI NY
OMIM
248600
3D Structure
ModBase 3D Structure for P21953
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.
Species
Human
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 BCKDHB 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
BCKDHB recombinant protein
Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems. Alternative splicing at this locus results in transcript variants with different 3 non-coding regions, but encoding the same isoform.
NCBI/Uniprot data below describe general gene information for BCKDHB. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
23,550 Da
NCBI Official Full Name
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform 1
NCBI Official Synonym Full Names
branched chain keto acid dehydrogenase E1 subunit beta
NCBI Official Symbol
BCKDHB  [Similar Products]
NCBI Official Synonym Symbols
E1B; BCKDE1B; BCKDH E1-beta
  [Similar Products]
NCBI Protein Information
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
UniProt Protein Name
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
UniProt Synonym Protein Names
Branched-chain alpha-keto acid dehydrogenase E1 component beta chain; BCKDE1B; BCKDH E1-beta
UniProt Gene Name
BCKDHB  [Similar Products]
UniProt Synonym Gene Names
BCKDE1B; BCKDH E1-beta  [Similar Products]
NCBI Summary for BCKDHB
This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation and feeding problems. Alternative splicing at this locus results in multiple transcript variants. [provided by RefSeq, Jan 2016]
UniProt Comments for BCKDHB
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2. It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
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.
Products associated with BCKDHB recombinant protein
 Reference Product  PubMed Publications
 BCKDHA recombinant protein  >15 publications with BCKDHB and BCKDHA
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