[Pyruvate dehydrogenase (acetyl-transferring)] kinase isozyme 1, mitochondrial

Name[Pyruvate dehydrogenase (acetyl-transferring)] kinase isozyme 1, mitochondrial
Synonyms
  • 2.7.11.2
  • PDH kinase 1
  • PDHK1
  • Pyruvate dehydrogenase kinase isoform 1
Gene NamePDK1
OrganismHuman
Amino acid sequence
>lcl|BSEQ0007401|[Pyruvate dehydrogenase (acetyl-transferring)] kinase isozyme 1, mitochondrial
MRLARLLRGAALAGPGPGLRAAGFSRSFSSDSGSSPASERGVPGQVDFYARFSPSPLSMK
QFLDFGSVNACEKTSFMFLRQELPVRLANIMKEISLLPDNLLRTPSVQLVQSWYIQSLQE
LLDFKDKSAEDAKAIYDFTDTVIRIRNRHNDVIPTMAQGVIEYKESFGVDPVTSQNVQYF
LDRFYMSRISIRMLLNQHSLLFGGKGKGSPSHRKHIGSINPNCNVLEVIKDGYENARRLC
DLYYINSPELELEELNAKSPGQPIQVVYVPSHLYHMVFELFKNAMRATMEHHANRGVYPP
IQVHVTLGNEDLTVKMSDRGGGVPLRKIDRLFNYMYSTAPRPRVETSRAVPLAGFGYGLP
ISRLYAQYFQGDLKLYSLEGYGTDAVIYIKALSTDSIERLPVYNKAAWKHYNTNHEADDW
CVPSREPKDMTTFRSA
Number of residues436
Molecular Weight49243.765
Theoretical pI9.05
GO Classification
Functions
  • pyruvate dehydrogenase (acetyl-transferring) kinase activity
  • ATP binding
  • protein kinase activity
Processes
  • protein phosphorylation
  • pyruvate metabolic process
  • regulation of acetyl-CoA biosynthetic process from pyruvate
  • mitophagy in response to mitochondrial depolarization
  • regulation of glucose metabolic process
  • intrinsic apoptotic signaling pathway in response to oxidative stress
  • hypoxia-inducible factor-1alpha signaling pathway
  • cell proliferation
  • small molecule metabolic process
  • glucose metabolic process
  • cellular metabolic process
Components
  • mitochondrial pyruvate dehydrogenase complex
  • mitochondrion
  • mitochondrial matrix
General FunctionPyruvate dehydrogenase (acetyl-transferring) kinase activity
Specific FunctionKinase that plays a key role in regulation of glucose and fatty acid metabolism and homeostasis via phosphorylation of the pyruvate dehydrogenase subunits PDHA1 and PDHA2. This inhibits pyruvate dehydrogenase activity, and thereby regulates metabolite flux through the tricarboxylic acid cycle, down-regulates aerobic respiration and inhibits the formation of acetyl-coenzyme A from pyruvate. Plays an important role in cellular responses to hypoxia and is important for cell proliferation under hypoxia. Protects cells against apoptosis in response to hypoxia and oxidative stress.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein ID1088281
UniProtKB IDQ15118
UniProtKB Entry NamePDK1_HUMAN
Cellular LocationMitochondrion matrix
Gene sequence
>lcl|BSEQ0012693|[Pyruvate dehydrogenase (acetyl-transferring)] kinase isozyme 1, mitochondrial (PDK1)
ATGAGGCTGGCGCGGCTGCTTCGCGGAGCCGCCTTGGCCGGCCCGGGCCCGGGGCTGCGC
GCCGCCGGCTTCAGCCGCAGCTTCAGCTCGGACTCGGGCTCCAGCCCGGCGTCCGAGCGC
GGCGTTCCGGGCCAGGTGGACTTCTACGCGCGCTTCTCGCCGTCCCCGCTCTCCATGAAG
CAGTTCCTGGACTTCGGATCAGTGAATGCTTGTGAAAAGACCTCATTTATGTTTCTGCGG
CAAGAGTTGCCTGTCAGACTGGCAAATATAATGAAAGAAATAAGTCTCCTTCCAGATAAT
CTTCTCAGGACACCATCCGTTCAATTGGTACAAAGCTGGTATATCCAGAGTCTTCAGGAG
CTTCTTGATTTTAAGGACAAAAGTGCTGAGGATGCTAAAGCTATTTATGAAAGGCCTAGA
AGAACATGGTTGCAGGTCTCTAGTTTATGCTGTATGGCCTGCAAGATGATCTTTACAGAT
ACTGTGATACGGATCAGAAACCGACACAATGATGTCATTCCCACAATGGCCCAGGGTGTG
ATTGAATACAAGGAGAGCTTTGGGGTGGATCCTGTCACCAGCCAGAATGTTCAGTACTTT
TTGGATCGATTCTACATGAGTCGCATTTCAATTAGAATGTTACTCAATCAGCACTCTTTA
TTGTTTGGTGGAAAAGGCAAAGGAAGTCCATCTCATCGAAAACACATTGGAAGCATAAAT
CCAAACTGCAATGTACTTGAAGTTATTAAAGATGGCTATGAAAATGCTAGGCGTCTGTGT
GATTTGTATTATATTAACTCTCCCGAACTAGAACTTGAAGAACTAAATGCAAAATCACCA
GGACAGCCAATACAAGTGGTTTATGTACCATCCCATCTCTATCACATGGTGTTTGAACTT
TTCAAGAATGCAATGAGAGCCACTATGGAACACCATGCCAACAGAGGTGTTTACCCCCCT
ATTCAAGTTCATGTCACGCTGGGTAATGAGGATTTGACTGTGAAGATGAGTGACCGAGGA
GGTGGCGTTCCTTTGAGGAAAATTGACAGACTTTTCAACTACATGTATTCAACTGCACCA
AGACCTCGTGTTGAGACCTCCCGCGCAGTGCCTCTGGCTGGTTTTGGTTATGGATTGCCC
ATATCACGTCTTTACGCACAATACTTCCAAGGAGACCTGAAGCTGTATTCCCTAGAGGGT
TACGGGACAGATGCAGTTATCTACATTAAGGCTCTGTCAACAGACTCAATAGAAAGACTC
CCAGTGTATAACAAAGCTGCCTGGAAGCATTACAACACCAACCACGAGGCTGATGACTGG
TGCGTCCCCAGCAGAGAACCCAAAGACATGACGACGTTCCGCAGTGCCTAG
GenBank Gene IDL42450
GeneCard IDNot Available
GenAtlas IDNot Available
HGNC IDHGNC:8809
Chromosome Location2
Locus2q31.1
References
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