NameKetohexokinase
Synonyms
  • 2.7.1.3
  • Hepatic fructokinase
Gene NameKHK
OrganismHuman
Amino acid sequence
>lcl|BSEQ0037299|Ketohexokinase
MEEKQILCVGLVVLDVISLVDKYPKEDSEIRCLSQRWQRGGNASNSCTVLSLLGAPCAFM
GSMAPGHVADFLVADFRRRGVDVSQVAWQSKGDTPSSCCIINNSNGNRTIVLHDTSLPDV
SATDFEKVDLTQFKWIHIEGRNASEQVKMLQRIDAHNTRQPPEQKIRVSVEVEKPREELF
QLFGYGDVVFVSKDVAKHLGFQSAEEALRGLYGRVRKGAVLVCAWAEEGADALGPDGKLL
HSDAFPPPRVVDTLGAGDTFNASVIFSLSQGRSVQEALRFGCQVAGKKCGLQGFDGIV
Number of residues298
Molecular Weight32522.765
Theoretical pINot Available
GO Classification
Functions
  • ATP binding
  • ketohexokinase activity
Processes
  • carbohydrate phosphorylation
  • small molecule metabolic process
  • carbohydrate metabolic process
  • response to insulin
  • response to zinc ion
  • fructose metabolic process
  • response to glucose
  • response to sucrose
  • fructose catabolic process to hydroxyacetone phosphate and glyceraldehyde-3-phosphate
  • response to fructose
Components
  • cytoplasm
  • cytosol
  • extracellular exosome
General FunctionKetohexokinase activity
Specific FunctionCatalyzes the phosphorylation of the ketose sugar fructose to fructose-1-phosphate.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein IDNot Available
UniProtKB IDP50053
UniProtKB Entry NameKHK_HUMAN
Cellular LocationNot Available
Gene sequence
>lcl|BSEQ0013972|Ketohexokinase (KHK)
ATGGAAGAGAAGCAGATCCTGTGCGTGGGGCTAGTGGTGCTGGACGTCATCAGCCTGGTG
GACAAGTACCCTAAGGAGGACTCGGAGATAAGGTGTTTGTCCCAGAGATGGCAGCGCGGA
GGCAACGCGTCCAACTCCTGCACCGTTCTCTCCCTGCTCGGAGCCCCCTGTGCCTTCATG
GGCTCAATGGCTCCTGGCCATGTTGCTGATTTTGTCCTGGATGACCTCCGCCGCTATTCT
GTGGACCTACGCTACACAGTCTTTCAGACCACAGGCTCCGTCCCCATCGCCACGGTCATC
ATCAACGAGGCCAGTGGTAGCCGCACCATCCTATACTATGACAGGAGCCTGCCAGATGTG
TCTGCTACAGACTTTGAGAAGGTTGATCTGACCCAGTTCAAGTGGATCCACATTGAGGGC
CGGAACGCATCGGAGCAGGTGAAGATGCTGCAGCGGATAGACGCACACAACACCAGGCAG
CCTCCAGAGCAGAAGATCCGGGTGTCCGTGGAGGTGGAGAAGCCACGAGAGGAGCTCTTC
CAGCTGTTTGGCTACGGAGACGTGGTGTTTGTCAGCAAAGATGTGGCCAAGCACTTGGGG
TTCCAGTCAGCAGAGGAAGCCTTGAGGGGCTTGTATGGTCGTGTGAGGAAAGGGGCTGTG
CTTGTCTGTGCCTGGGCTGAGGAGGGCGCCGACGCCCTGGGCCCTGATGGCAAATTGCTC
CACTCGGATGCTTTCCCGCCACCCCGCGTGGTGGATACACTGGGAGCTGGAGACACCTTC
AATGCCTCCGTCATCTTCAGCCTCTCCCAGGGGAGGAGCGTGCAGGAAGCACTGAGATTC
GGGTGCCAGGTGGCCGGCAAGAAGTGTGGCCTGCAGGGCTTTGATGGCATCGTGTGA
GenBank Gene IDNot Available
GeneCard IDNot Available
GenAtlas IDNot Available
HGNC IDHGNC:6315
Chromosome Location2
LocusNot Available
References
  1. Bonthron DT, Brady N, Donaldson IA, Steinmann B: Molecular basis of essential fructosuria: molecular cloning and mutational analysis of human ketohexokinase (fructokinase). Hum Mol Genet. 1994 Sep;3(9):1627-31. 7833921
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  4. Hayward BE, Bonthron DT: Structure and alternative splicing of the ketohexokinase gene. Eur J Biochem. 1998 Oct 1;257(1):85-91. 9799106
  5. Bian Y, Song C, Cheng K, Dong M, Wang F, Huang J, Sun D, Wang L, Ye M, Zou H: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J Proteomics. 2014 Jan 16;96:253-62. doi: 10.1016/j.jprot.2013.11.014. Epub 2013 Nov 22. 24275569
  6. Trinh CH, Asipu A, Bonthron DT, Phillips SE: Structures of alternatively spliced isoforms of human ketohexokinase. Acta Crystallogr D Biol Crystallogr. 2009 Mar;65(Pt 3):201-11. doi: 10.1107/S0907444908041115. Epub 2009 Feb 20. 19237742
  7. Asipu A, Hayward BE, O'Reilly J, Bonthron DT: Properties of normal and mutant recombinant human ketohexokinases and implications for the pathogenesis of essential fructosuria. Diabetes. 2003 Sep;52(9):2426-32. 12941785