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Identification
HMDB Protein ID HMDBP01205
Secondary Accession Numbers
  • 6501
Name Ketohexokinase
Synonyms
  1. Hepatic fructokinase
Gene Name KHK
Protein Type Enzyme
Biological Properties
General Function Carbohydrate transport and metabolism
Specific Function Not Available
Pathways
  • Fructose and Mannose Degradation
  • Fructose and mannose metabolism
  • Fructose intolerance, hereditary
  • fructose metabolism
  • Fructosuria
Reactions
Adenosine triphosphate + D-Fructose → ADP + D-fructose 1-phosphate details
Adenosine triphosphate + Sedoheptulose → ADP + Sedoheptulose 1-phosphate details
GO Classification
Biological Process
small molecule metabolic process
response to zinc ion
fructose catabolic process
response to fructose stimulus
response to insulin stimulus
response to glucose stimulus
response to sucrose stimulus
Cellular Component
cytosol
Molecular Function
ketohexokinase activity
ATP binding
Cellular Location Not Available
Gene Properties
Chromosome Location 2
Locus 2p23.3
SNPs KHK
Gene Sequence
>897 bp
ATGGAAGAGAAGCAGATCCTGTGCGTGGGGCTAGTGGTGCTGGACGTCATCAGCCTGGTG
GACAAGTACCCTAAGGAGGACTCGGAGATAAGGTGTTTGTCCCAGAGATGGCAGCGCGGA
GGCAACGCGTCCAACTCCTGCACCGTTCTCTCCCTGCTCGGAGCCCCCTGTGCCTTCATG
GGCTCAATGGCTCCTGGCCATGTTGCTGATTTTGTCCTGGATGACCTCCGCCGCTATTCT
GTGGACCTACGCTACACAGTCTTTCAGACCACAGGCTCCGTCCCCATCGCCACGGTCATC
ATCAACGAGGCCAGTGGTAGCCGCACCATCCTATACTATGACAGGAGCCTGCCAGATGTG
TCTGCTACAGACTTTGAGAAGGTTGATCTGACCCAGTTCAAGTGGATCCACATTGAGGGC
CGGAACGCATCGGAGCAGGTGAAGATGCTGCAGCGGATAGACGCACACAACACCAGGCAG
CCTCCAGAGCAGAAGATCCGGGTGTCCGTGGAGGTGGAGAAGCCACGAGAGGAGCTCTTC
CAGCTGTTTGGCTACGGAGACGTGGTGTTTGTCAGCAAAGATGTGGCCAAGCACTTGGGG
TTCCAGTCAGCAGAGGAAGCCTTGAGGGGCTTGTATGGTCGTGTGAGGAAAGGGGCTGTG
CTTGTCTGTGCCTGGGCTGAGGAGGGCGCCGACGCCCTGGGCCCTGATGGCAAATTGCTC
CACTCGGATGCTTTCCCGCCACCCCGCGTGGTGGATACACTGGGAGCTGGAGACACCTTC
AATGCCTCCGTCATCTTCAGCCTCTCCCAGGGGAGGAGCGTGCAGGAAGCACTGAGATTC
GGGTGCCAGGTGGCCGGCAAGAAGTGTGGCCTGCAGGGCTTTGATGGCATCGTGTGA
Protein Properties
Number of Residues 298
Molecular Weight 32730.055
Theoretical pI 5.93
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Ketohexokinase
MEEKQILCVGLVVLDVISLVDKYPKEDSEIRCLSQRWQRGGNASNSCTVLSLLGAPCAFM
GSMAPGHVADFVLDDLRRYSVDLRYTVFQTTGSVPIATVIINEASGSRTILYYDRSLPDV
SATDFEKVDLTQFKWIHIEGRNASEQVKMLQRIDAHNTRQPPEQKIRVSVEVEKPREELF
QLFGYGDVVFVSKDVAKHLGFQSAEEALRGLYGRVRKGAVLVCAWAEEGADALGPDGKLL
HSDAFPPPRVVDTLGAGDTFNASVIFSLSQGRSVQEALRFGCQVAGKKCGLQGFDGIV
GenBank ID Protein 4557693
UniProtKB/Swiss-Prot ID P50053
UniProtKB/Swiss-Prot Entry Name KHK_HUMAN
PDB IDs
GenBank Gene ID NM_000221.2
GeneCard ID KHK
GenAtlas ID KHK
HGNC ID HGNC:6315
References
General References
  1. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]
  2. 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. [PubMed:7833921 ]
  3. Hayward BE, Bonthron DT: Structure and alternative splicing of the ketohexokinase gene. Eur J Biochem. 1998 Oct 1;257(1):85-91. [PubMed:9799106 ]
  4. 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. [PubMed:19237742 ]