Page last updated: 2024-09-28

ALDOB Deficiency

An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity, resulting in accumulation of fructose-1-phosphate. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycemia following ingestion of fructose. Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. Patients develop a strong distaste for sweet food, and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet.

Synonyms(10)

Synonym
Fructose-1,6-Bisphosphate Aldolase B Deficiency
Fructose-1,6-Biphosphate Aldolase Deficiency
Aldolase B Deficiency
ALDOB Deficiency
Fructose Aldolase B Deficiency
Fructose Intolerance
Fructose-1-Phosphate Aldolase Deficiency
Fructosemia
Hereditary Fructose Intolerance
Fructose Intolerance, Hereditary

Research Excerpts

Overview

ExcerptReference
"Undiscovered fructose intolerance is more likely the younger the patient is, whereas the frequency of glucose intolerance increases with age."( Ahnefeld, FW; Bässler, KH; Grünert, A; Halmágyi, M; Mehnert, H; Schmitz, JE, 1987)
"Hereditary fructose intolerance is due to a deficiency of liver aldolase (aldolase B)."( Delpech, M; Dreyfus, JC; Grégori, C; Kahn, A; Schapira, F, 1982)
"Fructose intolerance is more prevalent with fructose alone than with HFCS in health and in IBS."( Bharucha, AE; Skoog, SM; Zinsmeister, AR, 2008)
"Fructose intolerance is another jigsaw piece of the IBS puzzle that may respond to dietary modification."( Choi, YK; Jackson, M; Kraft, N; Rao, SS; Zimmerman, B, 2008)
"Hereditary fructose intolerance is a rare, but potentially lethal, inherited disorder of fructose metabolism, caused by mutation of the aldolase B gene."( Bouteldja, N; Timson, DJ, 2010)

Treatment

ExcerptReference
"Hereditary fructose intolerance (HFI) was diagnosed in a 61 year-old male patient on account of liver dysfunction followed by prolonged shock immediately after the administration of a fructose and lactose infusion postoperatively."( Balogh, D; Decristoforo, A; Dworzak, E; Hackl, JM; Kunz, F; Maier, F; Puschendorf, B, 1978)

Research

Studies (220)

TimeframeStudies, This Condition (%)All Conditions %
pre-1990105 (47.73)23.3326
1990's32 (14.55)12.5806
2000's30 (13.64)18.1394
2010's36 (16.36)28.8240
2020's17 (7.73)9.53
DrugIndicatedRelationship StrengthStudiesTrials
ammonium hydroxide0low50
methane0low20
3-hydroxybutyric acid0low10
methylmalonic acid0low10
lactic acid0low30
dihydroxyacetone0low10
glycerol0low10
hydrogen carbonate0low30
hydrogen0medium103
kynurenine0low10
pyridoxine0low10
uric acid0medium161
urea0low20
mercaptoethanol0low20
sorbitol0medium271
glutamine0low10
sucrose0low90
adenosine diphosphate0low10
galactose0low100
edetic acid0low10
tyrosine0low100
adenosine monophosphate0low10
leucine0low10
lactose0low100
methionine0low10
tryptophan0medium21
arginine0low20
xylitol0low10
glycidol0low10
uridine diphosphate galactose0low10
deuterium0low10
fluorides0low10
transferrin0low70
fructose-1-phosphate0medium81
deoxyglucose0low10
proline0low10
hawkinsin0low10
glycogen0low30
fructans0low10
D-fructopyranose0medium1488
cystine0low10
vitamin k semiquinone radical0low10
cytochalasin b0low10
phosphorus0medium61
phosphocreatine0medium11
nad0low10
glucagon0low30
oligonucleotides0low10
cellulose0low10
invert sugar0low10
fructose-1,6-diphosphate0low10
ascorbic acid0low20
vitamin b 120low20
technetium tc 99m dimercaptosuccinic acid0low10
sapropterin0low10
folic acid0low20

Protein Targets (409)

ProteinPotency MeasurementsInhibition MeasurementsActivation MeasurementsDrugs
Protein skinhead-10101
Monocarboxylate transporter 20101
Solute carrier family 22 member 200101
Solute carrier family 22 member 60101
Chain A, Heat Shock Protein 900011
Chain A, GLUTAMINE PHOSPHORIBOSYLPYROPHOSPHATE AMIDOTRANSFERASE0011
Chain B, GLUTAMINE PHOSPHORIBOSYLPYROPHOSPHATE AMIDOTRANSFERASE0011
Chain A, EOSINOPHIL-DERIVED NEUROTOXIN0101
Chain A, EOSINOPHIL-DERIVED NEUROTOXIN0101
Chain A, EOSINOPHIL-DERIVED NEUROTOXIN0101
Chain A, Myosin Ie Heavy Chain0011
Chain A, Preprotein translocase secA0011
Chain A, Ribonuclease pancreatic0101
Chain A, Ribonuclease pancreatic0101
Chain A, Ribonuclease pancreatic0101
Chain A, Ribonuclease pancreatic0101
Chain A, Ribonuclease pancreatic0101
Chain A, Phosphoribosylformylglycinamidine synthase0101
Chain A, nucleoside diphosphate kinase A0011
Chain B, nucleoside diphosphate kinase A0011
Chain D, DNA polymerase III subunit gamma0011
Chain D, DNA polymerase III subunit gamma0011
Chain A [Pyruvate dehydrogenase [lipoamide]] kinase isozyme 30011
Chain A [Pyruvate dehydrogenase [lipoamide]] kinase isozyme 30011
Chain A, Kinesin-like protein KIF110101
regulator of G-protein signaling 42002
HPr kinase/phosphorylase0011
Mitogen-activated protein kinase kinase kinase 70022
ATP-dependent molecular chaperone HSP820001
Heat shock protein HSP 90-alpha0113
Heat shock protein HSP 90-beta0113
2-dehydropantoate 2-reductase0224
Heat shock 70 kDa protein 1A 0112
Endoplasmic reticulum chaperone BiP0011
Heat shock cognate 71 kDa protein0011
Pyruvate kinase PKM 0001
Pyruvate kinase PKLR 0001
Endoplasmin0001
Heat shock cognate 71 kDa protein0011
5'-nucleotidase0101
Mu-type opioid receptor0011
Delta-type opioid receptor0011
Kappa-type opioid receptor0011
Endoplasmin0112
P2Y purinoceptor 20022
Phosphatidylinositol 4-kinase alpha0202
P2X purinoceptor 10011
P2Y purinoceptor 10022
P2Y purinoceptor 10022
P2X purinoceptor 10011
P2X purinoceptor 40011
P2X purinoceptor 50011
P2X purinoceptor 60011
P2X purinoceptor 30011
Inositol monophosphatase 11001
Heat shock protein 75 kDa, mitochondrial0101
P2Y purinoceptor 60011
Phosphatidylinositol 4-kinase type 2-beta0202
P2Y purinoceptor 110022
Phosphatidylinositol 4-kinase type 2-alpha0202
P2Y purinoceptor 120011
Sensor protein kinase WalK0101
Phosphatidylinositol 4-kinase beta0202
P2X purinoceptor 20011
Chain A, Glycogen Phosphorylase B0011
Chain B, Glycogen Phosphorylase B0011
Chain A, ADP-dependent glucokinase0101
Chain A, Ribonuclease pancreatic0101
Chain A, Ribonuclease pancreatic0101
Chain A, phosphodiesterase-nucleotide pyrophosphatase0101
Chain A, GLYCOGEN PHOSPHORYLASE B0011
Chain B, GLYCOGEN PHOSPHORYLASE B0011
Chain A, GLYCOGEN PHOSPHORYLASE B0011
RAR-related orphan receptor gamma6006
AR protein8008
glucocorticoid receptor [Homo sapiens]4004
estrogen-related nuclear receptor alpha8008
euchromatic histone-lysine N-methyltransferase 29009
v-jun sarcoma virus 17 oncogene homolog (avian)4004
2'-deoxynucleoside 5'-phosphate N-hydrolase 10101
2'-deoxynucleoside 5'-phosphate N-hydrolase 10101
5'-AMP-activated protein kinase subunit beta-20011
Transient receptor potential cation channel subfamily M member 20101
Alcohol dehydrogenase E chain0101
Alcohol dehydrogenase S chain0101
L-lactate dehydrogenase A chain0112
Adenylate kinase isoenzyme 10001
Fructose-1,6-bisphosphatase 10101
Alkaline phosphatase, tissue-nonspecific isozyme0203
Fructose-1,6-bisphosphatase 10101
Inosine-5'-monophosphate dehydrogenase0101
Cytochrome P450 2C9 0101
Proto-oncogene tyrosine-protein kinase Src0101
Amine oxidase [flavin-containing] B0101
5'-nucleotidase0001
Choline O-acetyltransferase0101
Adenylate kinase 2, mitochondrial0102
GTP:AMP phosphotransferase AK3, mitochondrial0001
Adenosine receptor A10011
Adenylate kinase isoenzyme 1 0102
5'-AMP-activated protein kinase subunit gamma-10022
5'-AMP-activated protein kinase catalytic subunit alpha-20011
Adenylate kinase 2, mitochondrial0001
Glycine--tRNA ligase0101
5'-AMP-activated protein kinase catalytic subunit alpha-10022
Adenosine deaminase-like protein0001
Protease 0112
Histamine H3 receptor0101
5'-AMP-activated protein kinase subunit gamma-30011
5'-AMP-activated protein kinase subunit gamma-20011
5'-AMP-activated protein kinase subunit beta-10022
ATP-dependent phosphofructokinase4004
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE4004
Chain E, LYSINE, ARGININE, ORNITHINE-BINDING PROTEIN0011
Chain E, LYSINE, ARGININE, ORNITHINE-BINDING PROTEIN0011
Chain E, LYSINE, ARGININE, ORNITHINE-BINDING PROTEIN0011
thioredoxin reductase5005
phosphopantetheinyl transferase3003
NFKB1 protein, partial1001
GLS protein3003
thyroid stimulating hormone receptor3003
alpha-galactosidase2002
cytochrome P450 2C19 precursor1001
chromobox protein homolog 17007
flap endonuclease 14004
DNA polymerase iota isoform a (long)5005
muscarinic acetylcholine receptor M11001
lethal factor (plasmid)4004
Cytochrome P450 3A40001
Nitric oxide synthase, endothelial0002
Nitric oxide synthase, brain0012
Nitric oxide synthase, brain 0001
Nitric oxide synthase, inducible0001
Nitric oxide synthase, inducible0013
Cationic amino acid transporter 30101
Chain A, Hyaluronidase, phage associated0101
acetylcholinesterase2002
GLI family zinc finger 36006
thioredoxin glutathione reductase4004
cytochrome P450 family 3 subfamily A polypeptide 42002
retinoic acid nuclear receptor alpha variant 18008
estrogen nuclear receptor alpha110011
vitamin D (1,25- dihydroxyvitamin D3) receptor4004
IDH13003
nuclear factor erythroid 2-related factor 2 isoform 23003
thyroid hormone receptor beta isoform 23003
nuclear factor erythroid 2-related factor 2 isoform 16006
lethal(3)malignant brain tumor-like protein 1 isoform I1001
geminin5005
lamin isoform A-delta107007
Polyphenol oxidase 20102
Pancreatic alpha-amylase0202
Albumin0101
Urease0101
Prolyl 4-hydroxylase subunit alpha-10001
Tyrosinase0101
Alpha-2B adrenergic receptor0011
Spike glycoprotein2002
Hyaluronate lyase0101
Prolyl hydroxylase EGLN20001
Egl nine homolog 10001
Prolyl hydroxylase EGLN30001
Hypoxia-inducible factor 1-alpha inhibitor0001
Solute carrier family 23 member 10101
glp-1 receptor, partial2002
TDP1 protein1001
Smad31001
PINK11001
67.9K protein1001
Parkin1001
bromodomain adjacent to zinc finger domain 2B2002
neutrophil cytosol factor 10101
NPC intracellular cholesterol transporter 1 precursor1001
mitogen-activated protein kinase 11001
ras-related protein Rab-9A1001
urokinase-type plasminogen activator precursor1001
plasminogen precursor1001
urokinase plasminogen activator surface receptor precursor1001
survival motor neuron protein isoform d1001
Solute carrier family 2, facilitated glucose transporter member 10101
Solute carrier family 2, facilitated glucose transporter member 30101
Solute carrier family 2, facilitated glucose transporter member 40101
Chain A, TYROSYL-DNA PHOSPHODIESTERASE3003
hypoxia-inducible factor 1 alpha subunit1001
nuclear receptor subfamily 1, group I, member 32002
progesterone receptor1001
Histone H2A.x2002
Eyes absent homolog 20101
Polyunsaturated fatty acid lipoxygenase ALOX15B1001
Voltage-dependent calcium channel gamma-2 subunit3003
Cellular tumor antigen p534004
Amyloid-beta precursor protein0101
Angiotensin-converting enzyme 0202
Glutamate receptor 23003
Histamine H2 receptor1001
Metallo-beta-lactamase VIM-130101
Beta-lactamase 0101
Beta-lactamase 0101
Beta-lactamase VIM-1 0101
Carbonic anhydrase 10205
Carbonic anhydrase 20317
Carbonic anhydrase 40204
Carbonic anhydrase0204
Carbonic anhydrase 150204
Chain A, Dihydrofolate reductase0011
Chain A, Putative fructose-1,6-bisphosphate aldolase2002
Chain A, HADH2 protein3003
Chain B, HADH2 protein3003
Chain A, 2-oxoglutarate Oxygenase4004
Chain A, Ferritin light chain3003
acid sphingomyelinase1001
USP1 protein, partial5005
aldehyde dehydrogenase 1 family, member A15005
retinoid X nuclear receptor alpha5005
glucocerebrosidase1001
activating transcription factor 64004
lysosomal alpha-glucosidase preproprotein1001
15-hydroxyprostaglandin dehydrogenase [NAD(+)] isoform 12002
DNA polymerase beta1001
Multidrug resistance-associated protein 40001
Bile salt export pump0303
Thymidylate synthase0202
Thymidylate synthase0101
Dihydrofolate reductase0011
Aldo-keto reductase family 1 member B10101
Alpha-synuclein1001
Folylpolyglutamate synthase, mitochondrial0001
Solute carrier organic anion transporter family member 1A30101
Folylpolyglutamate synthase, mitochondrial0001
Multidrug resistance associated protein0001
importin subunit beta-1 isoform 12002
snurportin-12002
DNA polymerase kappa isoform 12002
histone acetyltransferase KAT2A isoform 14004
Beta-galactosidase0101
Lipopolysaccharide heptosyltransferase 10101
PA-I galactophilic lectin0011
Jacalin0011
Chain A, Glutamine Binding Protein0011
15-lipoxygenase, partial1001
arylsulfatase A2002
Bloom syndrome protein isoform 12002
peripheral myelin protein 22 isoform 12002
histone-lysine N-methyltransferase 2A isoform 2 precursor2002
Neutral amino acid transporter A0202
Asc-type amino acid transporter 10101
Neutral amino acid transporter B(0)0202
Carbonic anhydrase-like protein, putative0003
Amino acid transporter0202
Chain B, EUKARYOTIC TRANSLATION INITIATION FACTOR 4E0011
peroxisome proliferator-activated receptor delta2002
thyroid hormone receptor beta isoform a1001
interleukin 81001
Chain A, Cruzipain1001
Albumin0101
Voltage-dependent L-type calcium channel subunit alpha-1C0101
Microtubule-associated protein tau2002
nuclear receptor ROR-gamma isoform 11001
estrogen receptor 2 (ER beta)1001
cytochrome P450 2D61001
thyroid stimulating hormone receptor2002
cellular tumor antigen p53 isoform a1001
Integrin beta-31001
Integrin alpha-IIb1001
Chain A, Lectin0011
Chain A, Lectin0011
Chain A, Lectin0011
Chain A, Lectin0011
Chain A, ERYTHRINA CRISTA-GALLI LECTIN0011
Chain A, ERYTHRINA CRISTA-GALLI LECTIN0011
Chain A, cellulase0011
Chain A, cellulase0011
Chain A, Galectin-30011
Chain A, Anti-tumor lectin0101
Galectin-30101
Galectin-90011
Galectin-80011
Beta-galactoside-binding lectin0101
Galectin-10113
Galectin-30112
Galectin-30113
Galectin-70011
Alpha 1,4 galactosyltransferase0001
Chain A, AMINOPEPTIDASE0101
Chain A, AMINOPEPTIDASE0101
Chain A, Leucine Aminopeptidase0101
Intestinal-type alkaline phosphatase0202
Large neutral amino acids transporter small subunit 10404
Proton-coupled amino acid transporter 10203
Phospholipase A-2-activating protein0202
Glutaminyl-peptide cyclotransferase0101
Chain A, Methionyl-tRNA synthetase0011
Chain A, Methionyl-tRNA synthetase0011
Chain A, Aminopeptidase0101
Chain A, Methionine aminopeptidase0101
S-ribosylhomocysteine lyase0101
Adenylate cyclase type 50001
S-adenosylmethionine synthase isoform type-10001
S-adenosylmethionine synthase isoform type-20001
Fatty acid synthase0101
Chain A, Beta-lactamase2002
Chain A, JmjC domain-containing histone demethylation protein 3A1001
peptidyl-prolyl cis-trans isomerase NIMA-interacting 11001
tumor susceptibility gene 101 protein1001
GTP-binding nuclear protein Ran isoform 11001
Guanine nucleotide-binding protein G1001
Serine racemase0101
Chain B, Exotoxin A0011
Chain B, Exotoxin A0011
Chain B, Exotoxin A0011
Glutamate 5-kinase0001
Glutamate receptor ionotropic, NMDA 1 0103
Pyridoxal kinase0001
Bile salt export pump0101
Chain A, MALTOPORIN0011
Chain B, MALTOPORIN0011
pregnane X nuclear receptor2002
peripheral myelin protein 222002
Carbonic anhydrase 90114
Gamma-aminobutyric acid receptor subunit pi0101
Gamma-aminobutyric acid receptor subunit alpha-10001
Gamma-aminobutyric acid receptor subunit beta-10001
Gamma-aminobutyric acid receptor subunit alpha-20001
Gamma-aminobutyric acid receptor subunit alpha-30001
Gamma-aminobutyric acid receptor subunit beta-10101
Gamma-aminobutyric acid receptor subunit delta0101
Gamma-aminobutyric acid receptor subunit gamma-20101
Gamma-aminobutyric acid receptor subunit alpha-50101
Gamma-aminobutyric acid receptor subunit alpha-30101
Gamma-aminobutyric acid receptor subunit alpha-40001
Gamma-aminobutyric acid receptor subunit gamma-20001
Gamma-aminobutyric acid receptor subunit gamma-10101
Gamma-aminobutyric acid receptor subunit alpha-20101
Sodium- and chloride-dependent GABA transporter 10101
Gamma-aminobutyric acid receptor subunit alpha-40101
Gamma-aminobutyric acid receptor subunit gamma-30101
Gamma-aminobutyric acid receptor subunit alpha-60101
Sodium- and chloride-dependent GABA transporter 20101
Sodium- and chloride-dependent GABA transporter 30101
Sodium- and chloride-dependent betaine transporter0101
Gamma-aminobutyric acid receptor subunit alpha-10101
Gamma-aminobutyric acid receptor subunit beta-30101
Gamma-aminobutyric acid receptor subunit beta-20101
Fatty-acid amide hydrolase 10101
GABA theta subunit0101
Gamma-aminobutyric acid receptor subunit epsilon0101
Chain A, Trp Rna-binding Attenuation Protein0011
Chain K, Trp Rna-binding Attenuation Protein0011
Chain B, tryptophanyl-tRNA synthetase0011
Chain C, Tryptophanyl-tRNA synthetase II0011
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)1001
peroxisome proliferator activated receptor gamma1001
aryl hydrocarbon receptor1001
M-phase phosphoprotein 81001
Carbonic anhydrase 120002
Dihydrofolate reductase0202
Plasma kallikrein0002
Myeloperoxidase0101
Carbonic anhydrase 30002
Stromelysin-10101
Indoleamine 2,3-dioxygenase 10102
Carbonic anhydrase 60002
Substance-P receptor0101
Carbonic anhydrase 5A, mitochondrial0002
Carbonic anhydrase 70002
Tryptophan 2,3-dioxygenase0102
Carbonic anhydrase0002
Glutamate receptor ionotropic, NMDA 2A 0002
Glutamate receptor ionotropic, NMDA 2B0002
Glutamate receptor ionotropic, NMDA 2C0002
SLC16A10 protein0002
Uracil nucleotide/cysteinyl leukotriene receptor0101
Carbonic anhydrase 0002
Tryprostatin B synthase0001
Carbonic anhydrase0002
Carbonic anhydrase0002
Glutamate receptor ionotropic, NMDA 2D0002
2-C-methyl-D-erythritol 2,4-cyclodiphosphate synthase0011
Carbonic anhydrase 0002
Glutamate receptor ionotropic, NMDA 3B0002
Monocarboxylate transporter 100002
Carbonic anhydrase 130001
Glutamate receptor ionotropic, NMDA 3A0002
Carbonic anhydrase 140002
Carbonic anhydrase 5B, mitochondrial0002
Chain A, CHORISMATE MUTASE0101
Chain A, TYROSYL-tRNA SYNTHETASE0011
Adenosine receptor A10001
Adenosine receptor A2a0001
Tubulin--tyrosine ligase0101
Taste receptor type 2 member 140001
caspase 7, apoptosis-related cysteine protease1001
caspase-31001
Rap guanine nucleotide exchange factor 32002
Urease subunit alpha0101
Urease subunit beta0101
Olfactory receptor 51E20011
Chain A, Glycogen phosphorylase, liver form0011
Chain A, glycogen phosphorylase, liver form0011
Chain A, Glycogen phosphorylase, liver form0011
Chain A, Glycogen phosphorylase, liver form0011
Solute carrier family 22 member 60001
Solute carrier family 22 member 80101
Solute carrier family 2, facilitated glucose transporter member 90112
Guanine deaminase0101
Beta-1,4-galactosyltransferase 1 0001
N-acetyllactosaminide alpha-1,3-galactosyltransferase0001
P2Y purinoceptor 140011
vitamin D3 receptor isoform VDRA1001
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]