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Protein

Fructose-bisphosphate aldolase B

Gene

ALDOB

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Miscellaneous

In vertebrates, 3 forms of this ubiquitous glycolytic enzyme are found, aldolase A in muscle, aldolase B in liver and aldolase C in brain.

Catalytic activityi

D-fructose 1,6-bisphosphate = glycerone phosphate + D-glyceraldehyde 3-phosphate.

Kineticsi

  1. KM=1.6 µM for fructose 1,6-bisphosphate1 Publication
  2. KM=2.3 mM for fructose 1-phosphate1 Publication

    Pathwayi: glycolysis

    This protein is involved in step 4 of the subpathway that synthesizes D-glyceraldehyde 3-phosphate and glycerone phosphate from D-glucose.
    Proteins known to be involved in the 4 steps of the subpathway in this organism are:
    1. no protein annotated in this organism
    2. Glucose-6-phosphate isomerase, Glucose-6-phosphate isomerase, Glucose-6-phosphate isomerase (GPI), Glucose-6-phosphate isomerase (GPI), Glucose-6-phosphate isomerase (GPI), Glucose-6-phosphate isomerase (GPI), Glucose-6-phosphate isomerase, Glucose-6-phosphate isomerase (GPI)
    3. ATP-dependent 6-phosphofructokinase (PFKM), ATP-dependent 6-phosphofructokinase, liver type (PFKL), ATP-dependent 6-phosphofructokinase, muscle type (PFKM), ATP-dependent 6-phosphofructokinase, platelet type (PFKP)
    4. Fructose-bisphosphate aldolase, Fructose-bisphosphate aldolase (ALDOB), Fructose-bisphosphate aldolase, Fructose-bisphosphate aldolase, Fructose-bisphosphate aldolase, Fructose-bisphosphate aldolase, Fructose-bisphosphate aldolase, Fructose-bisphosphate aldolase (ALDOA), Fructose-bisphosphate aldolase C (ALDOC), Fructose-bisphosphate aldolase (HEL-S-87p), Fructose-bisphosphate aldolase, Fructose-bisphosphate aldolase (ALDOA), Fructose-bisphosphate aldolase (ALDOC), Fructose-bisphosphate aldolase, Fructose-bisphosphate aldolase, Fructose-bisphosphate aldolase (ALDOB), Fructose-bisphosphate aldolase (ALDOA), Fructose-bisphosphate aldolase (ALDOA), Fructose-bisphosphate aldolase B (ALDOB), Fructose-bisphosphate aldolase A (ALDOA), Fructose-bisphosphate aldolase, Fructose-bisphosphate aldolase (ALDOC)
    This subpathway is part of the pathway glycolysis, which is itself part of Carbohydrate degradation.
    View all proteins of this organism that are known to be involved in the subpathway that synthesizes D-glyceraldehyde 3-phosphate and glycerone phosphate from D-glucose, the pathway glycolysis and in Carbohydrate degradation.

    Sites

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Binding sitei56Substrate1
    Binding sitei147Substrate1
    Active sitei188Proton acceptorHMP:1
    Active sitei230Schiff-base intermediate with dihydroxyacetone-P1
    Sitei364Necessary for preference for fructose 1,6-bisphosphate over fructose 1-phosphate1

    GO - Molecular functioni

    GO - Biological processi

    Keywordsi

    Molecular functionLyase
    Biological processGlycolysis
    LigandSchiff base

    Enzyme and pathway databases

    BioCyciMetaCyc:HS06234-MONOMER.
    BRENDAi4.1.2.13. 2681.
    ReactomeiR-HSA-5657560. Hereditary fructose intolerance.
    R-HSA-70171. Glycolysis.
    R-HSA-70263. Gluconeogenesis.
    R-HSA-70350. Fructose catabolism.
    SABIO-RKiP05062.
    SIGNORiP05062.
    UniPathwayiUPA00109; UER00183.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Fructose-bisphosphate aldolase B (EC:4.1.2.13)
    Alternative name(s):
    Liver-type aldolase
    Gene namesi
    Name:ALDOB
    Synonyms:ALDB
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    Proteomesi
    • UP000005640 Componenti: Chromosome 9

    Organism-specific databases

    EuPathDBiHostDB:ENSG00000136872.17.
    HGNCiHGNC:417. ALDOB.

    Subcellular locationi

    Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

    Keywords - Cellular componenti

    Cytoplasm, Cytoskeleton

    Pathology & Biotechi

    Involvement in diseasei

    Hereditary fructose intolerance (HFI)11 Publications
    The disease is caused by mutations affecting the gene represented in this entry.
    Disease descriptionAutosomal recessive disease that results in an inability to metabolize fructose and related sugars. Complete exclusion of fructose results in dramatic recovery; however, if not treated properly, HFI subjects suffer episodes of hypoglycemia, general ill condition, and risk of death the remainder of life.
    See also OMIM:229600
    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Natural variantiVAR_07534846R → W in HFI; reduced enzymatic activity. 1 PublicationCorresponds to variant dbSNP:rs41281039Ensembl.1
    Natural variantiVAR_02082274I → T in HFI; affects proper folding. 1 PublicationCorresponds to variant dbSNP:rs781023784Ensembl.1
    Natural variantiVAR_020823120 – 121Missing in HFI. 1 Publication2
    Natural variantiVAR_000551135C → R in HFI; America; partial activity. 1 Publication1
    Natural variantiVAR_000553150A → P in HFI; frequent mutation. 6 PublicationsCorresponds to variant dbSNP:rs1800546Ensembl.1
    Natural variantiVAR_000554175A → D in HFI; frequent mutation. 6 PublicationsCorresponds to variant dbSNP:rs76917243Ensembl.1
    Natural variantiVAR_058211178C → R in HFI. 1 Publication1
    Natural variantiVAR_020824185P → R in HFI. 1 Publication1
    Natural variantiVAR_020826222V → F in HFI; affects proper folding. 1 Publication1
    Natural variantiVAR_020827229L → P in HFI; affects proper folding. 1 Publication1
    Natural variantiVAR_000555257L → P in HFI; Italy. 3 PublicationsCorresponds to variant dbSNP:rs764701775Ensembl.1
    Natural variantiVAR_058212284L → P in HFI. 1 Publication1
    Natural variantiVAR_020828304R → Q in HFI; 100-fold decrease in catalytic efficiency for substrates FBP and F1P. 1 PublicationCorresponds to variant dbSNP:rs145078268Ensembl.1
    Natural variantiVAR_000556304R → W in HFI; Turkey; 4800-fold decrease in catalytic efficiency for FBP and inactive with F1P. 2 PublicationsCorresponds to variant dbSNP:rs555935217Ensembl.1
    Natural variantiVAR_000557335N → K in HFI; frequent mutation. 5 PublicationsCorresponds to variant dbSNP:rs78340951Ensembl.1
    Natural variantiVAR_000558338A → V in HFI; Turkey and South Europe. 2 PublicationsCorresponds to variant dbSNP:rs77718928Ensembl.1
    Natural variantiVAR_075349343Y → H in HFI; almost no effect on enzymatic activity at 30 degrees Celsius, but reduced activity at higher temperatures. 1 PublicationCorresponds to variant dbSNP:rs369586696Ensembl.1

    Keywords - Diseasei

    Disease mutation

    Organism-specific databases

    DisGeNETi229.
    MalaCardsiALDOB.
    MIMi229600. phenotype.
    OpenTargetsiENSG00000136872.
    Orphaneti469. Hereditary fructose intolerance.
    PharmGKBiPA24710.

    Chemistry databases

    DrugBankiDB02515. 3-Phosphoglycerol.

    Polymorphism and mutation databases

    BioMutaiALDOB.
    DMDMi113611.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Initiator methionineiRemovedHMP:1 Publication
    ChainiPRO_00002169402 – 364Fructose-bisphosphate aldolase BAdd BLAST363

    Amino acid modifications

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Modified residuei2N-acetylalanineHMP:1
    Modified residuei13N6-succinyllysineHMP:1
    Modified residuei36PhosphoserineND:1
    Modified residuei39PhosphothreonineND:1
    Modified residuei89PhosphoserineND:1
    Modified residuei119PhosphothreonineND:1
    Modified residuei121N6-succinyllysineHMP:1
    Modified residuei132PhosphoserineND:1
    Modified residuei272PhosphoserineND:1
    Modified residuei276PhosphoserineND:1
    Modified residuei299PhosphoserineHMP:1
    Modified residuei301PhosphoserineHMP:1
    Modified residuei309PhosphoserineND:1
    Modified residuei317N6-succinyllysineHMP:1

    Keywords - PTMi

    Acetylation, Phosphoprotein

    Proteomic databases

    EPDiP05062.
    MaxQBiP05062.
    PaxDbiP05062.
    PeptideAtlasiP05062.
    PRIDEiP05062.

    PTM databases

    iPTMnetiP05062.
    PhosphoSitePlusiP05062.

    Expressioni

    Gene expression databases

    BgeeiENSG00000136872.
    CleanExiHS_ALDOB.
    ExpressionAtlasiP05062. baseline and differential.
    GenevisibleiP05062. HS.

    Organism-specific databases

    HPAiCAB020827.
    HPA002198.

    Interactioni

    Subunit structurei

    Homotetramer. Interacts with BBS1, BBS2, BBS4 and BBS7.1 Publication

    Binary interactionsi

    Show more details

    GO - Molecular functioni

    • ATPase binding Source: BHF-UCL
    • cytoskeletal protein binding Source: BHF-UCL
    • identical protein binding Source: BHF-UCL

    Protein-protein interaction databases

    BioGridi106730. 16 interactors.
    IntActiP05062. 28 interactors.
    STRINGi9606.ENSP00000363988.

    Structurei

    Secondary structure

    1364
    Legend: HelixTurnBeta strandPDB Structure known for this area
    Show more details
    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Helixi10 – 23ND:14
    Turni25 – 27ND:3
    Beta strandi29 – 33ND:5
    Helixi37 – 46ND:10
    Helixi53 – 64ND:12
    Helixi68 – 72ND:5
    Beta strandi74 – 79ND:6
    Helixi83 – 85ND:3
    Turni89 – 91ND:3
    Helixi94 – 100ND:7
    Beta strandi104 – 108ND:5
    Beta strandi113 – 115ND:3
    Beta strandi119 – 121ND:3
    Beta strandi123 – 125ND:3
    Helixi131 – 140ND:10
    Beta strandi145 – 152ND:8
    Helixi161 – 179ND:19
    Turni180 – 182ND:3
    Beta strandi184 – 191ND:8
    Helixi199 – 219ND:21
    Helixi224 – 226ND:3
    Helixi246 – 260ND:15
    Beta strandi267 – 271ND:5
    Helixi277 – 289ND:13
    Beta strandi290 – 292ND:3
    Beta strandi296 – 303ND:8
    Helixi304 – 306ND:3
    Helixi308 – 314ND:7
    Helixi318 – 320ND:3
    Helixi321 – 338ND:18
    Turni339 – 341ND:3
    Helixi351 – 354ND:4

    3D structure databases

    Select the link destinations:
    PDBei
    RCSB PDBi
    PDBji
    Links Updated
    PDB entryMethodResolution (Å)ChainPositionsPDBsum
    1QO5X-ray2.50A/B/C/D/E/F/G/H/I/J/K/L/M/N/O/P/Q/R2-364[»]
    1XDLX-ray3.00A/B/C/D/W/X/Y/Z2-364[»]
    1XDMX-ray3.00A/B/C/D/W/X/Y/Z2-364[»]
    ProteinModelPortaliP05062.
    SMRiP05062.
    ModBaseiSearch...
    MobiDBiSearch...

    Miscellaneous databases

    EvolutionaryTraceiP05062.

    Family & Domainsi

    Sequence similaritiesi

    Phylogenomic databases

    eggNOGiKOG1557. Eukaryota.
    COG3588. LUCA.
    GeneTreeiENSGT00390000010235.
    HOGENOMiHOG000220876.
    HOVERGENiHBG002386.
    InParanoidiP05062.
    KOiK01623.
    OMAiPLCKPWA.
    OrthoDBiEOG091G0A9T.
    PhylomeDBiP05062.
    TreeFamiTF314203.

    Family and domain databases

    Gene3Di3.20.20.70. 1 hit.
    InterProiView protein in InterPro
    IPR029768. Aldolase_I_AS.
    IPR013785. Aldolase_TIM.
    IPR000741. FBA_I.
    PfamiView protein in Pfam
    PF00274. Glycolytic. 1 hit.
    PROSITEiView protein in PROSITE
    PS00158. ALDOLASE_CLASS_I. 1 hit.

    Sequencei

    Sequence statusi: Complete.

    Sequence processingi: The displayed sequence is further processed into a mature form.

    P05062-1 [UniParc]FASTAAdd to basket

    « Hide

            10         20         30         40         50
    MAHRFPALTQ EQKKELSEIA QSIVANGKGI LAADESVGTM GNRLQRIKVE
    60 70 80 90 100
    NTEENRRQFR EILFSVDSSI NQSIGGVILF HETLYQKDSQ GKLFRNILKE
    110 120 130 140 150
    KGIVVGIKLD QGGAPLAGTN KETTIQGLDG LSERCAQYKK DGVDFGKWRA
    160 170 180 190 200
    VLRIADQCPS SLAIQENANA LARYASICQQ NGLVPIVEPE VIPDGDHDLE
    210 220 230 240 250
    HCQYVTEKVL AAVYKALNDH HVYLEGTLLK PNMVTAGHAC TKKYTPEQVA
    260 270 280 290 300
    MATVTALHRT VPAAVPGICF LSGGMSEEDA TLNLNAINLC PLPKPWKLSF
    310 320 330 340 350
    SYGRALQASA LAAWGGKAAN KEATQEAFMK RAMANCQAAK GQYVHTGSSG
    360
    AASTQSLFTA CYTY
    Length:364
    Mass (Da):39,473
    Last modified:January 23, 2007 - v2
    Checksum:iDCE314E7AC5586CA
    GO

    Experimental Info

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Sequence conflicti54E → D in AAA51691 (PubMed:3016456).IKR:1
    Sequence conflicti250A → D in CAA25072 (PubMed:6689266).IKR:1
    Sequence conflicti278E → D in BAA00125 (PubMed:2830249).IKR:1
    Sequence conflicti309S → V no nucleotide entry (PubMed:6585824).IKR:1
    Sequence conflicti348S → C in BAA00125 (PubMed:2830249).IKR:1

    Natural variant

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Natural variantiVAR_07534846R → W in HFI; reduced enzymatic activity. 1 PublicationCorresponds to variant dbSNP:rs41281039Ensembl.1
    Natural variantiVAR_02082274I → T in HFI; affects proper folding. 1 PublicationCorresponds to variant dbSNP:rs781023784Ensembl.1
    Natural variantiVAR_020823120 – 121Missing in HFI. 1 Publication2
    Natural variantiVAR_038429134R → S. Corresponds to variant dbSNP:rs10123355Ensembl.1
    Natural variantiVAR_000551135C → R in HFI; America; partial activity. 1 Publication1
    Natural variantiVAR_000552148W → R in one subject with fructose intolerance; rare variant; America. 1 PublicationCorresponds to variant dbSNP:rs118204430Ensembl.1
    Natural variantiVAR_000553150A → P in HFI; frequent mutation. 6 PublicationsCorresponds to variant dbSNP:rs1800546Ensembl.1
    Natural variantiVAR_000554175A → D in HFI; frequent mutation. 6 PublicationsCorresponds to variant dbSNP:rs76917243Ensembl.1
    Natural variantiVAR_058211178C → R in HFI. 1 Publication1
    Natural variantiVAR_020824185P → R in HFI. 1 Publication1
    Natural variantiVAR_020825207E → Q. Corresponds to variant dbSNP:rs3739721Ensembl.1
    Natural variantiVAR_020826222V → F in HFI; affects proper folding. 1 Publication1
    Natural variantiVAR_020827229L → P in HFI; affects proper folding. 1 Publication1
    Natural variantiVAR_000555257L → P in HFI; Italy. 3 PublicationsCorresponds to variant dbSNP:rs764701775Ensembl.1
    Natural variantiVAR_038430268I → N. Corresponds to variant dbSNP:rs10989495Ensembl.1
    Natural variantiVAR_058212284L → P in HFI. 1 Publication1
    Natural variantiVAR_020828304R → Q in HFI; 100-fold decrease in catalytic efficiency for substrates FBP and F1P. 1 PublicationCorresponds to variant dbSNP:rs145078268Ensembl.1
    Natural variantiVAR_000556304R → W in HFI; Turkey; 4800-fold decrease in catalytic efficiency for FBP and inactive with F1P. 2 PublicationsCorresponds to variant dbSNP:rs555935217Ensembl.1
    Natural variantiVAR_000557335N → K in HFI; frequent mutation. 5 PublicationsCorresponds to variant dbSNP:rs78340951Ensembl.1
    Natural variantiVAR_000558338A → V in HFI; Turkey and South Europe. 2 PublicationsCorresponds to variant dbSNP:rs77718928Ensembl.1
    Natural variantiVAR_075349343Y → H in HFI; almost no effect on enzymatic activity at 30 degrees Celsius, but reduced activity at higher temperatures. 1 PublicationCorresponds to variant dbSNP:rs369586696Ensembl.1

    Sequence databases

    Select the link destinations:
    EMBLi
    GenBanki
    DDBJi
    Links Updated
    X02747 mRNA. Translation: CAA26526.1.
    D00183 Genomic DNA. Translation: BAA00125.1.
    M15656, M15657 Genomic DNA. Translation: AAA51691.1.
    AL353621 Genomic DNA. No translation available.
    CH471105 Genomic DNA. Translation: EAW58951.1.
    X00270 mRNA. Translation: CAA25072.1.
    X01098 mRNA. Translation: CAA25572.1.
    CCDSiCCDS6756.1.
    PIRiA41505. ADHUB.
    RefSeqiNP_000026.2. NM_000035.3.
    UniGeneiHs.530274.

    Genome annotation databases

    EnsembliENST00000374855; ENSP00000363988; ENSG00000136872.
    GeneIDi229.
    KEGGihsa:229.
    UCSCiuc004bbk.3. human.

    Keywords - Coding sequence diversityi

    Polymorphism

    Similar proteinsi

    Entry informationi

    Entry nameiALDOB_HUMAN
    AccessioniPrimary (citable) accession number: P05062
    Secondary accession number(s): Q13741, Q13742, Q5T7D6
    Entry historyiIntegrated into UniProtKB/Swiss-Prot: August 13, 1987
    Last sequence update: January 23, 2007
    Last modified: November 22, 2017
    This is version 198 of the entry and version 2 of the sequence. See complete history.
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program
    DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

    Miscellaneousi

    Keywords - Technical termi

    3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

    Documents

    1. Human chromosome 9
      Human chromosome 9: entries, gene names and cross-references to MIM
    2. Human entries with polymorphisms or disease mutations
      List of human entries with polymorphisms or disease mutations
    3. Human polymorphisms and disease mutations
      Index of human polymorphisms and disease mutations
    4. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    5. PATHWAY comments
      Index of metabolic and biosynthesis pathways
    6. PDB cross-references
      Index of Protein Data Bank (PDB) cross-references
    7. SIMILARITY comments
      Index of protein domains and families