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Branched chain ketoacid dehydrogenase kinase

BCKD kinase, BCKDK, Branched-chain alpha-ketoacid dehydrogenase-kinase, branched-chain ketoacid dehydrogenase kinase
mitochondrial kinase; involved in oxidative degradation of branched-chain amino acids [RGD, Feb 2006] (from NCBI)
Top mentioned proteins: ACID, BCAA, CAN, STEP, mTOR
Papers on BCKD kinase
Impaired adiponectin signaling contributes to disturbed catabolism of branched-chain amino acids in diabetic mice.
Tao et al., Xi'an, China. In Diabetes, 2015
Interestingly, in adiponectin (APN) knockout (APN(-/-)) mice fed with a high-fat diet (HD), PP2Cm expression and BCKD activity were significantly decreased, whereas BCKD kinase (BDK), which inhibits BCKD activity, was markedly increased.
Branched-chain amino acid metabolism: from rare Mendelian diseases to more common disorders.
Review
Lee et al., Houston, United States. In Hum Mol Genet, 2014
The regulation of the BCKDC by the branched-chain ketoacid dehydrogenase kinase has also been implicated in a new inborn error of metabolism characterized by autism, intellectual disability and seizures.
Two novel mutations in the BCKDK (branched-chain keto-acid dehydrogenase kinase) gene are responsible for a neurobehavioral deficit in two pediatric unrelated patients.
Agulló et al., Barcelona, Spain. In Hum Mutat, 2014
Inactivating mutations in the BCKDK gene, which codes for the kinase responsible for the negative regulation of the branched-chain α-keto acid dehydrogenase complex (BCKD), have recently been associated with a form of autism in three families.
PGC-1α-mediated branched-chain amino acid metabolism in the skeletal muscle.
Miura et al., Tokyo, Japan. In Plos One, 2013
The expression of BCKDH kinase (BCKDK), which phosphorylates BCKDH and suppresses its enzymatic activity, was unchanged.
Structure-based design and mechanisms of allosteric inhibitors for mitochondrial branched-chain α-ketoacid dehydrogenase kinase.
Chuang et al., Dallas, United States. In Proc Natl Acad Sci U S A, 2013
BCAA homeostasis is controlled by the mitochondrial branched-chain α-ketoacid dehydrogenase complex (BCKDC), which is negatively regulated by the specific BCKD kinase (BDK).
A study of molecular changes relating to energy metabolism and cellular stress in people with Huntington's disease: looking for biomarkers.
Anuszewska et al., Warsaw, Poland. In J Bioenerg Biomembr, 2013
The comparison revealed statistically significant Global Pattern Recognition Fold Change (FC) for 6 mRNA transcripts, reflecting an increase of: MAOB (FC = 3.07; p = 0.0005) which encodes an outer mitochondrial membrane-bound enzyme called monoamine oxidase type B; TGM2 (FC = 1.8; p = 0.02) encoding a transglutaminase 2 that mediates cellular stress; SLC2A4 (FC = 1.64; p = 0.02) solute carrier family 2 (facilitated glucose transporter) member 4; branched chain ketoacid dehydrogenase kinase (BCKDK) (FC = 1.34; p = 0.02); decrease of LDHA (FC = -1.16;
Oral leucine supplementation is sensed by the brain but neither reduces food intake nor induces an anorectic pattern of gene expression in the hypothalamus.
Donato et al., São Paulo, Brazil. In Plos One, 2012
We assessed the hypothalamic gene expression and observed that leucine supplementation increased the expression of enzymes (BCAT1, BCAT2 and BCKDK) that metabolize branched-chain amino acids.
Mutations in BCKD-kinase lead to a potentially treatable form of autism with epilepsy.
Impact
Gleeson et al., San Diego, United States. In Science, 2012
We have identified inactivating mutations in the gene BCKDK (Branched Chain Ketoacid Dehydrogenase Kinase) in consanguineous families with autism, epilepsy, and intellectual disability.
Tissue-specific and nutrient regulation of the branched-chain α-keto acid dehydrogenase phosphatase, protein phosphatase 2Cm (PP2Cm).
Sun et al., Shanghai, China. In J Biol Chem, 2012
Furthermore, we have established that PP2Cm interacts with the BCKD E2 subunit and competes with the BCKD kinase in a substrate-dependent and mutually exclusive manner.
Positive selection in the chromosome 16 VKORC1 genomic region has contributed to the variability of anticoagulant response in humans.
Sabbagh et al., Paris, France. In Plos One, 2011
The most extreme scores of the different selection tests are found within a smaller 45 kb region that contains VKORC1 and three other genes (BCKDK, MYST1 (KAT8), and PRSS8) with different functions.
Protein phosphatase 2Cm is a critical regulator of branched-chain amino acid catabolism in mice and cultured cells.
Wang et al., Los Angeles, United States. In J Clin Invest, 2009
BCKD is inhibited by phosphorylation of its E1alpha subunit at Ser293, which is catalyzed by BCKD kinase.
Obesity-related elevations in plasma leucine are associated with alterations in enzymes involved in branched-chain amino acid metabolism.
Lynch et al., Penn Hills, United States. In Am J Physiol Endocrinol Metab, 2007
Whereas no obesity-related changes were observed in rodent skeletal muscle BCATm, pS293, or total BCKD E1alpha or BCKD kinase, in liver BCKD E1alpha was either unaltered or diminished by obesity, and pS293 (associated with the inactive state of BCKD) increased, along with BCKD kinase.
Inhibition of PI3-kinase signaling by glucocorticoids results in increased branched-chain amino acid degradation in renal epithelial cells.
Price et al., Atlanta, United States. In Am J Physiol Cell Physiol, 2007
This response was achieved, in part, by alterations in the transcription of BCKD subunits and BCKD kinase, which inactivates the enzyme complex by phosphorylation.
A novel branched-chain amino acid metabolon. Protein-protein interactions in a supramolecular complex.
Hutson et al., Winston-Salem, United States. In J Biol Chem, 2007
The BCKD complex is organized around a cubic core consisting of 24 lipoate-bearing dihydrolipoyl transacylase (E2) subunits, associated with the branched-chain alpha-keto acid decarboxylase/dehydrogenase (E1), dihydrolipoamide dehydrogenase (E3), BCKD kinase, and BCKD phosphatase.
Impaired growth and neurological abnormalities in branched-chain alpha-keto acid dehydrogenase kinase-deficient mice.
GeneRIF
Harris et al., Indianapolis, United States. In Biochem J, 2006
branched-chain a-keto acid dehydrogenase complex kinase plays an important role in conserving branched-chain amino acids by limiting their oxidation
Tissue-specific translation of murine branched-chain alpha-ketoacid dehydrogenase kinase mRNA is dependent upon an upstream open reading frame in the 5'-untranslated region.
GeneRIF
Danner et al., Atlanta, United States. In J Biol Chem, 2004
Tissue-specific translation of murine BCKDK mRNA is dependent upon an upstream open reading frame in the 5'-untranslated region
Mitochondrial alpha-ketoacid dehydrogenase kinases: a new family of protein kinases.
Review
Harris et al., Indianapolis, United States. In Adv Second Messenger Phosphoprotein Res, 1996
Arg288 is required for recognition by BCKDK of the phosphorylation site on the E1alpha subunit of the BCKDH complex.
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