Recombinant Human [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial (BCKDK)

Code CSB-YP002607HU
MSDS
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Source Yeast
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Code CSB-EP002607HU
MSDS
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Source E.coli
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Code CSB-EP002607HU-B
MSDS
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Source E.coli
Conjugate Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
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Code CSB-BP002607HU
MSDS
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Source Baculovirus
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Code CSB-MP002607HU
MSDS
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Source Mammalian cell
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Product Details

Purity
>85% (SDS-PAGE)
Target Names
BCKDK
Uniprot No.
Alternative Names
[3 methyl 2 oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial ; [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase; BCKD kinase ; BCKD-kinase; BCKD_HUMAN; BCKDHKIN; Bckdk; BCKDKD; BDK; Branched chain alpha keto acid dehydrogenase kinase; Branched chain ketoacid dehydrogenase kinase; Branched-chain alpha-ketoacid dehydrogenase kinase; mitochondrial
Species
Homo sapiens (Human)
Expression Region
31-412
Target Protein Sequence
STSATDTHHV EMARERSKTV TSFYNQSAID AAAEKPSVRL TPTMMLYAGR SQDGSHLLKS ARYLQQELPV RIAHRIKGFR CLPFIIGCNP TILHVHELYI RAFQKLTDFP PIKDQADEAQ YCQLVRQLLD DHKDVVTLLA EGLRESRKHI EDEKLVRYFL DKTLTSRLGI RMLATHHLAL HEDKPDFVGI ICTRLSPKKI IEKWVDFARR LCEHKYGNAP RVRINGHVAA RFPFIPMPLD YILPELLKNA MRATMESHLD TPYNVPDVVI TIANNDVDLI IRISDRGGGI AHKDLDRVMD YHFTTAEAST QDPRISPLFG HLDMHSGAQS GPMHGFGFGL PTSRAYAEYL GGSLQLQSLQ GIGTDVYLRL RHIDGREESF RI
Protein Length
Full Length of Mature Protein
Tag Info
Tag type will be determined during the manufacturing process.
The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
Form
Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
Buffer before Lyophilization
Tris/PBS-based buffer, 6% Trehalose, pH 8.0
Reconstitution
We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
Troubleshooting and FAQs
Storage Condition
Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life
The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Lead Time
Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
Notes
Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet
Please contact us to get it.

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Target Background

Function
Catalyzes the phosphorylation and inactivation of the branched-chain alpha-ketoacid dehydrogenase complex, the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Key enzyme that regulate the activity state of the BCKD complex.
Gene References into Functions
  1. our study indicates that BCKDK promotes colorectal tumorigenesis through up-regulation of the MAPK signaling pathway by phosphorylating MEK at Ser221. PMID: 28501528
  2. There is a relationship between plamsa CST and BCKDK in sepsis patients. PMID: 27773658
  3. Results demonstrate that BCKDK mutations can result in neurobehavioral deficits in humans. PMID: 24449431
  4. This publication is about the mouse gene but contains the 5' sequence of the human gene, including upstream open reading frame information. PMID: 15302860
  5. employment of multidimensional heteronuclear NMR techniques to determine the structure and dynamics of the LBD of the human branched-chain alpha-keto acid dehydrogenase complex (hbLBD) PMID: 11839747

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Involvement in disease
Branched-chain ketoacid dehydrogenase kinase deficiency (BCKDKD)
Subcellular Location
Mitochondrion matrix. Mitochondrion.
Protein Families
PDK/BCKDK protein kinase family
Tissue Specificity
Ubiquitous.
Database Links

HGNC: 16902

OMIM: 614901

KEGG: hsa:10295

STRING: 9606.ENSP00000219794

UniGene: Hs.513520

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