Search results for KHK

Showing 11 results out of 11

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Species

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Species

Types

Compartments

Reaction types

Search properties

Protein (3 results from a total of 3)

KHK

Identifier: R-HSA-70329
Species: Homo sapiens
Compartment: cytosol
Primary external reference: UniProt: KHK: P50053
Identifier: R-HSA-5656462
Species: Homo sapiens
Compartment: cytosol
Primary external reference: UniProt: KHK: P50053
Identifier: R-HSA-5656458
Species: Homo sapiens
Compartment: cytosol
Primary external reference: UniProt: P50053

Interactor (1 results from a total of 1)

KHK

Identifier: P50053-2
Species: Homo sapiens
Primary external reference: UniProt: P50053-2

Complex (3 results from a total of 3)

Identifier: R-HSA-3006523
Species: Homo sapiens
Compartment: cytosol
Identifier: R-HSA-5656448
Species: Homo sapiens
Compartment: cytosol
Identifier: R-HSA-5656443
Species: Homo sapiens
Compartment: cytosol

Set (1 results from a total of 1)

Identifier: R-HSA-5656460
Species: Homo sapiens
Compartment: cytosol

Reaction (2 results from a total of 2)

Identifier: R-HSA-5656459
Species: Homo sapiens
Compartment: cytosol
Variant KHK (ketohexokinase) protein fails to catalyze the phosphorylation of fructose to yield fructose 1-phosphate (Fru 1-P), the first step of fructose catabolism in the liver. This defect is associated with essential fructosuria, a rare benign condition characterized by elevated urinary fructose levels associated with consumption of fructose. Two missense mutant alleles have been identified in DNA sequencing studies of affected individuals (Bouthron et al. 1994). One, G40R, has no detectable activity. The second, A43T, encodes a protein whose liver ("A") isoform is inactive but whose peripheral ("C") isoform, though thermally unstable, retains some activity (Asipu et al. 2003).
Identifier: R-HSA-70333
Species: Homo sapiens
Compartment: cytosol
Cytosolic ketohexokinase (KHK, also known as fructokinase) catalyzes the reaction of D-fructose (Fru) and ATP to form D-fructose 1-phosphate (Fru 1-P) and ADP. Two isoforms of the enzyme, A and C, are encoded by alternatively spliced forms of the gene; both form catalytically active dimers. The C isoform is predominant in liver and kidney tissues, has high affinity for fructose, and is probably responsible for the bulk of fructose phosphorylation in vivo (Asipu et al. 2003; Trinh et al. 2009). The A isoform is found in lower levels in many other tissues and may serve a role in fructose metabolism outside of liver and kidney (Funari et al. 2005). The physiological role of KHK has been established from metabolic and DNA sequencing studies of patients with essential fructosuria (Bonthron et al. 1994) and in mouse models for this disease (Diggle et al. 2010; Ishimoto et al. 2012).

Pathway (1 results from a total of 1)

Identifier: R-HSA-5657562
Species: Homo sapiens
Deficiencies in KHK (ketohexokinase) are associated with essential fructosuria (Bonthron et al. 1994).
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