Fructose Metabolism, Inborn Errors

Inherited abnormalities of fructose metabolism, which include three known autosomal recessive types: hepatic fructokinase deficiency (essential fructosuria), hereditary fructose intolerance, and hereditary fructose-1,6-diphosphatase deficiency. Essential fructosuria is a benign asymptomatic metabolic disorder caused by deficiency in fructokinase, leading to decreased conversion of fructose to fructose-1-phosphate and alimentary hyperfructosemia, but with no clinical dysfunction; may produce a false-positive diabetes test.

Synonym
Fructose Metabolism, Inborn Errors

Timeframe	Studies, This Condition (%)	All Conditions %
pre-1990	20 (51.28)	23.3326
1990's	11 (28.21)	12.5806
2000's	2 (5.13)	18.1394
2010's	6 (15.38)	28.8240
2020's	0 (0.00)	9.53

Drug	Relationship Strength	Studies	Trials
hydrogen	medium	1	1
uric acid	low	1	0
sorbitol	low	4	0
serine	low	1	0
sucrose	low	1	0
galactose	low	5	0
lactose	low	4	0
copper gluconate	low	1	0
mannose	low	2	0
fructose-1-phosphate	medium	1	1
fructose-6-phosphate	low	1	0
xylose	low	1	0
cortisone	low	1	0
glycogen	low	1	0
sorbose	low	1	0
arabinose	low	1	0
D-fructopyranose	medium	30	1
vitamin k semiquinone radical	low	1	0
xylulose	low	2	0
phosphorus	medium	1	1
phosphocreatine	medium	1	1
ribose	low	1	0
acebutolol	low	1	0
ascorbic acid	low	1	0

Protein Targets (184)

Protein	Potency Measurements	Inhibition Measurements	Activation Measurements	Drugs
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE	3	0	0	3
Chain A, Hyaluronidase, phage associated	0	1	0	1
acetylcholinesterase	1	0	0	1
RAR-related orphan receptor gamma	3	0	0	3
GLI family zinc finger 3	2	0	0	2
AR protein	2	0	0	2
thioredoxin glutathione reductase	2	0	0	2
cytochrome P450 family 3 subfamily A polypeptide 4	1	0	0	1
glucocorticoid receptor [Homo sapiens]	2	0	0	2
retinoic acid nuclear receptor alpha variant 1	2	0	0	2
estrogen-related nuclear receptor alpha	1	0	0	1
estrogen nuclear receptor alpha	4	0	0	4
vitamin D (1,25- dihydroxyvitamin D3) receptor	3	0	0	3
IDH1	2	0	0	2
euchromatic histone-lysine N-methyltransferase 2	3	0	0	3
chromobox protein homolog 1	6	0	0	6
nuclear factor erythroid 2-related factor 2 isoform 2	1	0	0	1
thyroid hormone receptor beta isoform 2	1	0	0	1
nuclear factor erythroid 2-related factor 2 isoform 1	2	0	0	2
lethal(3)malignant brain tumor-like protein 1 isoform I	1	0	0	1
geminin	5	0	0	5
lethal factor (plasmid)	2	0	0	2
lamin isoform A-delta10	2	0	0	2
Polyphenol oxidase 2	0	1	0	1
Pancreatic alpha-amylase	0	2	0	2
Albumin	0	1	0	1
Urease	0	1	0	1
Prolyl 4-hydroxylase subunit alpha-1	0	0	0	1
Tyrosinase	0	1	0	1
Alpha-2B adrenergic receptor	0	0	1	1
Spike glycoprotein	1	0	0	1
Hyaluronate lyase	0	1	0	1
Prolyl hydroxylase EGLN2	0	0	0	1
Egl nine homolog 1	0	0	0	1
Prolyl hydroxylase EGLN3	0	0	0	1
Hypoxia-inducible factor 1-alpha inhibitor	0	0	0	1
Solute carrier family 23 member 1	0	1	0	1
Chain A, HADH2 protein	1	0	0	1
Chain B, HADH2 protein	1	0	0	1
endonuclease IV	1	0	0	1
dopamine D1 receptor	1	0	0	1
thioredoxin reductase	2	0	0	2
pregnane X receptor	1	0	0	1
GLS protein	1	0	0	1
TDP1 protein	2	0	0	2
aldehyde dehydrogenase 1 family, member A1	2	0	0	2
progesterone receptor	1	0	0	1
regulator of G-protein signaling 4	1	0	0	1
retinoid X nuclear receptor alpha	1	0	0	1
pregnane X nuclear receptor	3	0	0	3
peroxisome proliferator-activated receptor delta	1	0	0	1
peroxisome proliferator activated receptor gamma	1	0	0	1
nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105), isoform CRA_a	1	0	0	1
v-jun sarcoma virus 17 oncogene homolog (avian)	1	0	0	1
peripheral myelin protein 22 isoform 1	1	0	0	1
atrial natriuretic peptide receptor 1 precursor	1	0	0	1
heat shock protein beta-1	1	0	0	1
flap endonuclease 1	2	0	0	2
survival motor neuron protein isoform d	1	0	0	1
cytochrome P450 3A4 isoform 1	1	0	0	1
M-phase phosphoprotein 8	1	0	0	1
muscarinic acetylcholine receptor M1	2	0	0	2
Gamma-aminobutyric acid receptor subunit pi	1	0	0	1
Bile salt export pump	0	3	0	3
Glucocorticoid receptor	0	2	0	2
Sex hormone-binding globulin	0	0	1	1
Glycine receptor subunit alpha-1	0	2	0	2
Corticosteroid-binding globulin	0	1	0	1
Gamma-aminobutyric acid receptor subunit beta-1	1	0	0	1
Gamma-aminobutyric acid receptor subunit delta	1	0	0	1
Gamma-aminobutyric acid receptor subunit gamma-2	1	0	0	1
Gamma-aminobutyric acid receptor subunit alpha-5	1	0	0	1
Gamma-aminobutyric acid receptor subunit alpha-3	1	0	0	1
Glycine receptor subunit beta	0	2	0	2
Glycine receptor subunit alpha-2	0	2	0	2
Gamma-aminobutyric acid receptor subunit gamma-1	1	0	0	1
Gamma-aminobutyric acid receptor subunit alpha-2	1	0	0	1
Glycine receptor subunit alpha-3	0	2	0	2
Gamma-aminobutyric acid receptor subunit alpha-4	1	0	0	1
Gamma-aminobutyric acid receptor subunit gamma-3	1	0	0	1
Gamma-aminobutyric acid receptor subunit alpha-6	1	0	0	1
Gamma-aminobutyric acid receptor subunit alpha-1	1	0	0	1
Gamma-aminobutyric acid receptor subunit beta-3	1	0	0	1
Gamma-aminobutyric acid receptor subunit beta-2	1	0	0	1
Potassium voltage-gated channel subfamily A member 1	0	0	1	1
TAR DNA-binding protein 43	2	0	0	2
GABA theta subunit	1	0	0	1
Multidrug and toxin extrusion protein 1	0	1	0	1
Gamma-aminobutyric acid receptor subunit epsilon	1	0	0	1
USP1 protein, partial	2	0	0	2
importin subunit beta-1 isoform 1	1	0	0	1
snurportin-1	1	0	0	1
DNA polymerase kappa isoform 1	3	0	0	3
histone acetyltransferase KAT2A isoform 1	2	0	0	2
Beta-galactosidase	0	1	0	1
Lipopolysaccharide heptosyltransferase 1	0	1	0	1
PA-I galactophilic lectin	0	0	1	1
Jacalin	0	0	1	1
Chain A, Lectin	0	0	1	1
Chain A, Lectin	0	0	1	1
Chain A, Lectin	0	0	1	1
Chain A, Lectin	0	0	1	1
Chain A, ERYTHRINA CRISTA-GALLI LECTIN	0	0	1	1
Chain A, ERYTHRINA CRISTA-GALLI LECTIN	0	0	1	1
Chain A, cellulase	0	0	1	1
Chain A, cellulase	0	0	1	1
Chain A, Galectin-3	0	0	1	1
Chain A, Anti-tumor lectin	0	1	0	1
Galectin-3	0	1	0	1
Galectin-9	0	0	1	1
Galectin-8	0	0	1	1
Beta-galactoside-binding lectin	0	1	0	1
Galectin-1	0	1	1	3
Galectin-3	0	1	1	2
Galectin-3	0	1	1	3
Galectin-7	0	0	1	1
Alpha 1,4 galactosyltransferase	0	0	0	1
transcriptional regulator ERG isoform 3	1	0	0	1
Alpha-mannosidase 2C1	0	1	0	1
Fucose-binding lectin PA-IIL	0	1	0	1
CD209 antigen	0	1	0	1
thyroid stimulating hormone receptor	1	0	0	1
EWS/FLI fusion protein	1	0	0	1
Thioredoxin reductase	0	1	0	1
Histone H2A.x	1	0	0	1
vitamin D3 receptor isoform VDRA	2	0	0	2
thyroid hormone receptor beta isoform a	1	0	0	1
mitogen-activated protein kinase 1	1	0	0	1
muscleblind-like protein 1 isoform 1	1	0	0	1
ATP-binding cassette sub-family C member 3	0	1	0	1
Multidrug resistance-associated protein 4	0	1	0	1
Flavodoxin	0	0	0	1
Voltage-dependent L-type calcium channel subunit alpha-1F	0	1	0	1
Cellular tumor antigen p53	1	0	0	1
Voltage-dependent L-type calcium channel subunit alpha-1D	0	1	0	1
Voltage-dependent L-type calcium channel subunit alpha-1S	0	1	0	1
Voltage-dependent L-type calcium channel subunit alpha-1C	0	1	0	1
Lanosterol 14-alpha demethylase	0	1	0	1
Canalicular multispecific organic anion transporter 1	0	1	0	1
Chain A, 2-oxoglutarate Oxygenase	1	0	0	1
phosphopantetheinyl transferase	1	0	0	1
Acetylcholinesterase	0	1	0	1
Pyruvate kinase PKM	0	0	1	1
Neutral amino acid transporter A	0	1	0	1
Cholinesterase	0	1	0	1
Neutral amino acid transporter B(0)	0	1	0	1
Proton-coupled amino acid transporter 1	0	1	0	1
Amino acid transporter	0	1	0	1
Chain A, MALTOPORIN	0	0	1	1
Chain B, MALTOPORIN	0	0	1	1
peripheral myelin protein 22	2	0	0	2
Carbonic anhydrase 2	0	1	1	2
Carbonic anhydrase 9	0	1	1	2
Chain A, Glycogen phosphorylase, liver form	0	0	1	1
Chain A, glycogen phosphorylase, liver form	0	0	1	1
Chain A, Glycogen phosphorylase, liver form	0	0	1	1
Chain A, Glycogen phosphorylase, liver form	0	0	1	1
Solute carrier family 22 member 6	0	0	0	1
Solute carrier family 22 member 8	0	1	0	1
Solute carrier family 2, facilitated glucose transporter member 9	0	1	1	2
Guanine deaminase	0	1	0	1
Albumin	0	0	1	1
Alpha-2A adrenergic receptor	0	1	0	1
60 kDa heat shock protein, mitochondrial	0	1	0	1
Alpha-1B adrenergic receptor	0	1	0	1
Alpha-2B adrenergic receptor	0	1	0	1
Alpha-2C adrenergic receptor	0	1	0	1
Alpha-2A adrenergic receptor	0	1	0	1
Alpha-1D adrenergic receptor	0	1	0	1
Sodium-dependent serotonin transporter	0	1	0	1
Alpha-1A adrenergic receptor	0	1	0	1
10 kDa heat shock protein, mitochondrial	0	1	0	1
D-alanyl-D-alanine dipeptidase	0	1	0	1
Thiosulfate sulfurtransferase	0	1	0	1
60 kDa chaperonin	0	1	0	1
10 kDa chaperonin	0	1	0	1
Transporter	0	1	0	1
Chain A, Beta-lactamase	1	0	0	1
Chain A, Ferritin light chain	1	0	0	1
DNA polymerase iota isoform a (long)	1	0	0	1
Rap guanine nucleotide exchange factor 3	1	0	0	1
Hsf1 protein	0	0	1	1
estrogen receptor 2 (ER beta)	1	0	0	1
Aldo-keto reductase family 1 member B1	0	0	0	1
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]

Fructose Metabolism, Inborn Errors

Synonyms(1)

Research

Studies (39)

Protein Targets (184)

Fructose Metabolism, Inborn Errors

Synonyms(1)

Research

Studies (39)

Drugs Studied (28)

Protein Targets (184)