Free for academic non-profit institutions. Other users need a Commercial license

Aliases for FBP1 Gene

Aliases for FBP1 Gene

  • Fructose-Bisphosphatase 1 2 3 5
  • D-Fructose-1,6-Bisphosphate 1-Phosphohydrolase 1 3 4
  • Liver FBPase 3 4
  • EC 3.1.3.11 4 63
  • FBPase 1 3 4
  • FBP 3 4
  • Fructose-1,6-Bisphosphatase 1 2
  • Growth-Inhibiting Protein 17 3
  • EC 3.1.3 63

External Ids for FBP1 Gene

Previous HGNC Symbols for FBP1 Gene

  • FBP

Previous GeneCards Identifiers for FBP1 Gene

  • GC09M088192
  • GC09M089093
  • GC09M090664
  • GC09M092706
  • GC09M094444
  • GC09M096405
  • GC09M097365
  • GC09M066974

Summaries for FBP1 Gene

Entrez Gene Summary for FBP1 Gene

  • Fructose-1,6-bisphosphatase 1, a gluconeogenesis regulatory enzyme, catalyzes the hydrolysis of fructose 1,6-bisphosphate to fructose 6-phosphate and inorganic phosphate. Fructose-1,6-diphosphatase deficiency is associated with hypoglycemia and metabolic acidosis. [provided by RefSeq, Jul 2008]

GeneCards Summary for FBP1 Gene

FBP1 (Fructose-Bisphosphatase 1) is a Protein Coding gene. Diseases associated with FBP1 include fructose-1,6-bisphosphatase deficiency and metabolic acidosis. Among its related pathways are Metabolism and Glycosaminoglycan metabolism. GO annotations related to this gene include identical protein binding and phosphoric ester hydrolase activity. An important paralog of this gene is FBP2.

UniProtKB/Swiss-Prot for FBP1 Gene

  • Catalyzes the hydrolysis of fructose 1,6-bisphosphate to fructose 6-phosphate in the presence of divalent cations, acting as a rate-limiting enzyme in gluconeogenesis. Plays a role in regulating glucose sensing and insulin secretion of pancreatic beta-cells. Appears to modulate glycerol gluconeogenesis in liver. Important regulator of appetite and adiposity; increased expression of the protein in liver after nutrient excess increases circulating satiety hormones and reduces appetite-stimulating neuropeptides and thus seems to provide a feedback mechanism to limit weight gain.

No data available for Tocris Summary , Gene Wiki entry , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for FBP1 Gene

Genomics for FBP1 Gene

Regulatory Elements for FBP1 Gene

Promoters for FBP1 Gene
Ensembl Regulatory Elements (ENSRs) TSS Distance (bp) Size (bp) Binding Sites for Transcription Factors within promoters

ENSRs around FBP1 on UCSC Golden Path with GeneCards custom track

Transcription factor binding sites by QIAGEN in the FBP1 gene promoter:

Genomic Location for FBP1 Gene

Chromosome:
9
Start:
94,603,133 bp from pter
End:
94,640,249 bp from pter
Size:
37,117 bases
Orientation:
Minus strand

Genomic View for FBP1 Gene

Genes around FBP1 on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
FBP1 Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
Genomic Location for FBP1 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for FBP1 Gene

Proteins for FBP1 Gene

  • Protein details for FBP1 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    P09467-F16P1_HUMAN
    Recommended name:
    Fructose-1,6-bisphosphatase 1
    Protein Accession:
    P09467
    Secondary Accessions:
    • O75571
    • Q53F94
    • Q96E46

    Protein attributes for FBP1 Gene

    Size:
    338 amino acids
    Molecular mass:
    36842 Da
    Cofactor:
    Name=Mg(2+); Xref=ChEBI:CHEBI:18420;
    Quaternary structure:
    • Homotetramer.
    SequenceCaution:
    • Sequence=AAC50207.1; Type=Erroneous gene model prediction; Evidence={ECO:0000305};

    Three dimensional structures from OCA and Proteopedia for FBP1 Gene

neXtProt entry for FBP1 Gene

Proteomics data for FBP1 Gene at MOPED

Post-translational modifications for FBP1 Gene

  • Modification sites at PhosphoSitePlus
  • Modification sites at neXtProt

Other Protein References for FBP1 Gene

Antibody Products

  • Cell Signaling Technology (CST) Antibodies for FBP1 (FBPase)

Domains & Families for FBP1 Gene

Gene Families for FBP1 Gene

Graphical View of Domain Structure for InterPro Entry

P09467

UniProtKB/Swiss-Prot:

F16P1_HUMAN :
  • Belongs to the FBPase class 1 family.
Family:
  • Belongs to the FBPase class 1 family.
genes like me logo Genes that share domains with FBP1: view

Function for FBP1 Gene

Molecular function for FBP1 Gene

GENATLAS Biochemistry:
fructose 1,6-bisphosphatase,hepatic,expressed in liver,fructose metabolism,neoglucogenesis,energy pathway
UniProtKB/Swiss-Prot BiophysicochemicalProperties:
Kinetic parameters: KM=2.7 uM for fructose 1,6-biphosphate {ECO:0000269 PubMed:17350621}; Note=The kinetic constants are determined for the recombinant enzyme expressed in E.coli.;
UniProtKB/Swiss-Prot CatalyticActivity:
D-fructose 1,6-bisphosphate + H(2)O = D-fructose 6-phosphate + phosphate.
UniProtKB/Swiss-Prot EnzymeRegulation:
Subject to complex allosteric regulation. The enzyme can assume an active R-state, or an inactive T-state. Intermediate conformations may exist. AMP acts as allosteric inhibitor. AMP binding affects the turnover of bound substrate and not the affinity for substrate. Fructose 2,6-bisphosphate acts as competitive inhibitor. Fructose 2,6-bisphosphate and AMP have synergistic effects.
UniProtKB/Swiss-Prot Function:
Catalyzes the hydrolysis of fructose 1,6-bisphosphate to fructose 6-phosphate in the presence of divalent cations, acting as a rate-limiting enzyme in gluconeogenesis. Plays a role in regulating glucose sensing and insulin secretion of pancreatic beta-cells. Appears to modulate glycerol gluconeogenesis in liver. Important regulator of appetite and adiposity; increased expression of the protein in liver after nutrient excess increases circulating satiety hormones and reduces appetite-stimulating neuropeptides and thus seems to provide a feedback mechanism to limit weight gain.
UniProtKB/Swiss-Prot Induction:
Up-regulated in pancreatic islets of individuals with type 2 diabetes.

Enzyme Numbers (IUBMB) for FBP1 Gene

Gene Ontology (GO) - Molecular Function for FBP1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005515 protein binding IPI 8387495
GO:0042802 identical protein binding IPI 16169070
genes like me logo Genes that share ontologies with FBP1: view
genes like me logo Genes that share phenotypes with FBP1: view

Human Phenotype Ontology for FBP1 Gene

HPO Id HPO Name Alternative Ids Definition Synonyms

Animal Model Products

miRNA for FBP1 Gene

miRTarBase miRNAs that target FBP1

No data available for Animal Models , Transcription Factor Targets and HOMER Transcription for FBP1 Gene

Localization for FBP1 Gene

Subcellular locations from

COMPARTMENTS
Jensen Localization Image for FBP1 Gene COMPARTMENTS Subcellular localization image for FBP1 gene
Compartment Confidence
cytosol 5
extracellular 5
chloroplast 3
nucleus 2
lysosome 1
mitochondrion 1
peroxisome 1
vacuole 1

No data available for Subcellular locations from UniProtKB/Swiss-Prot and Gene Ontology (GO) - Cellular Components for FBP1 Gene

Pathways & Interactions for FBP1 Gene

genes like me logo Genes that share pathways with FBP1: view

Pathways by source for FBP1 Gene

UniProtKB/Swiss-Prot P09467-F16P1_HUMAN

  • Pathway: Carbohydrate biosynthesis; gluconeogenesis.

Gene Ontology (GO) - Biological Process for FBP1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005975 carbohydrate metabolic process IEA --
GO:0006000 fructose metabolic process TAS 7558035
GO:0006094 gluconeogenesis TAS --
GO:0006111 regulation of gluconeogenesis IMP 7558035
GO:0044281 small molecule metabolic process TAS --
genes like me logo Genes that share ontologies with FBP1: view

No data available for SIGNOR curated interactions for FBP1 Gene

Drugs & Compounds for FBP1 Gene

(12) Drugs for FBP1 Gene - From: Novoseek, DrugBank, and DGIdb

Name Status Disease Links Group Role Mechanism of Action Clinical Trials
Adenosine monophosphate Approved Nutra Target, antagonist, inhibitory allosteric modulator 0
{4-[3-(6,7-Diethoxy-Quinazolin-4-Ylamino)-Phenyl]-Thiazol-2-Yl}-Methanol Experimental Pharma Target 0
2,5-Anhydroglucitol-1,6-Biphosphate Experimental Pharma Target 0
2,5-DICHLORO-N-(5-CHLORO-1,3-BENZOXAZOL-2-YL)BENZENESULFONAMIDE Experimental Pharma Target 0
2,5-DICHLORO-N-[5-METHOXY-7-(6-METHOXYPYRIDIN-3-YL)-1,3-BENZOXAZOL-2-YL]BENZENESULFONAMIDE Experimental Pharma Target 0

(9) Additional Compounds for FBP1 Gene - From: HMDB and Novoseek

Name Synonyms Role CAS Number PubChem IDs PubMed IDs
beta-D-Fructose 1,6-bisphosphate
Beta-D-Fructose 6-phosphate
  • beta-D-Fructose 6-phosphate
Fructose 1,6-bisphosphate
  • D-Fructose 1,6-biphosphate
  • D-Fructose 1,6-bis(dihydrogen phosphate)
  • D-Fructose 1,6-bisphosphate
  • D-Fructose 1,6-diphosphate
  • D-Fructose-1,6-bisphosphate
488-69-7
Fructose 6-phosphate
  • D-Fructose 6-phosphate
  • D-Fructose 6-phosphorate
  • D-Fructose 6-phosphoric acid
  • D-Fructose-6-P
  • D-Fructose-6-phosphate
643-13-0
Phosphate
  • NFB Orthophosphate
  • O-Phosphoric acid
  • Ortho-phosphate
  • Orthophosphate (PO43-)
  • Orthophosphate(3-)
14265-44-2
genes like me logo Genes that share compounds with FBP1: view

Transcripts for FBP1 Gene

Unigene Clusters for FBP1 Gene

Fructose-1,6-bisphosphatase 1:
Representative Sequences:

Alternative Splicing Database (ASD) splice patterns (SP) for FBP1 Gene

ExUns: 1a · 1b ^ 2a · 2b · 2c ^ 3a · 3b ^ 4 ^ 5a · 5b ^ 6a · 6b ^ 7a · 7b ^ 8a · 8b · 8c
SP1: - -
SP2: -
SP3: - -
SP4: -
SP5:

Relevant External Links for FBP1 Gene

GeneLoc Exon Structure for
FBP1
ECgene alternative splicing isoforms for
FBP1

Expression for FBP1 Gene

mRNA expression in normal human tissues for FBP1 Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

mRNA differential expression in normal tissues according to GTEx for FBP1 Gene

This gene is overexpressed in Liver (x22.2) and Kidney - Cortex (x5.7).

Protein differential expression in normal tissues from HIPED for FBP1 Gene

This gene is overexpressed in Liver, secretome (32.0) and Liver (11.8).

Integrated Proteomics: protein expression in normal tissues and cell lines from ProteomicsDB, PaxDb, MOPED, and MaxQB for FBP1 Gene



SOURCE GeneReport for Unigene cluster for FBP1 Gene Hs.494496

mRNA Expression by UniProt/SwissProt for FBP1 Gene

P09467-F16P1_HUMAN
Tissue specificity: Expressed in pancreatic islets.
genes like me logo Genes that share expression patterns with FBP1: view

Primer Products

In Situ Assay Products

No data available for Protein tissue co-expression partners for FBP1 Gene

Orthologs for FBP1 Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for FBP1 Gene

Organism Taxonomy Gene Similarity Type Details
chimpanzee
(Pan troglodytes)
Mammalia FBP1 36
  • 99 (a)
OneToOne
FBP1 35
  • 99.31 (n)
  • 99.11 (a)
cow
(Bos Taurus)
Mammalia FBP1 36
  • 88 (a)
OneToOne
FBP1 35
  • 85.11 (n)
  • 88.17 (a)
dog
(Canis familiaris)
Mammalia FBP1 35
  • 87.18 (n)
  • 90.53 (a)
FBP1 36
  • 91 (a)
OneToOne
mouse
(Mus musculus)
Mammalia Fbp1 16
Fbp1 36
  • 85 (a)
OneToOne
Fbp1 35
  • 83.63 (n)
  • 84.91 (a)
oppossum
(Monodelphis domestica)
Mammalia -- 36
  • 70 (a)
OneToMany
-- 36
  • 89 (a)
OneToMany
-- 36
  • 63 (a)
OneToMany
platypus
(Ornithorhynchus anatinus)
Mammalia -- 36
  • 90 (a)
ManyToMany
rat
(Rattus norvegicus)
Mammalia Fbp1 35
  • 83.83 (n)
  • 85.21 (a)
chicken
(Gallus gallus)
Aves FBP1 35
  • 74.36 (n)
  • 80.47 (a)
FBP1 36
  • 65 (a)
OneToOne
lizard
(Anolis carolinensis)
Reptilia FBP1 36
  • 70 (a)
OneToOne
African clawed frog
(Xenopus laevis)
Amphibia fbp-prov 35
tropical clawed frog
(Silurana tropicalis)
Amphibia fbp1 35
  • 72.78 (n)
  • 82.25 (a)
MGC75708 35
zebrafish
(Danio rerio)
Actinopterygii fbp1a 36
  • 80 (a)
OneToMany
fbp1b 36
  • 80 (a)
OneToMany
fbp1a 35
  • 73.23 (n)
  • 80 (a)
wufk92h02 35
fruit fly
(Drosophila melanogaster)
Insecta fbp 37
  • 64 (a)
fbp 36
  • 59 (a)
OneToMany
worm
(Caenorhabditis elegans)
Secernentea fbp-1 36
  • 59 (a)
OneToMany
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_AFR593C 35
  • 57.34 (n)
  • 50 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes FBP1 35
  • 52.17 (n)
  • 48.45 (a)
FBP1 36
  • 43 (a)
OneToMany
FBP1 38
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes KLLA0E01211g 35
  • 52.73 (n)
  • 48.92 (a)
soybean
(Glycine max)
eudicotyledons Gma.2522 35
barley
(Hordeum vulgare)
Liliopsida Hv.5733 35
rice
(Oryza sativa)
Liliopsida Os.11756 35
bread mold
(Neurospora crassa)
Ascomycetes NCU04797 35
  • 57.23 (n)
  • 46.99 (a)
fission yeast
(Schizosaccharomyces pombe)
Schizosaccharomycetes fbp1 35
  • 50.21 (n)
  • 45.99 (a)
Species with no ortholog for FBP1:
  • Actinobacteria (Mycobacterium tuberculosis)
  • African malaria mosquito (Anopheles gambiae)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • beta proteobacteria (Neisseria meningitidis)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rainbow trout (Oncorhynchus mykiss)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea squirt (Ciona intestinalis)
  • sea squirt (Ciona savignyi)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • thale cress (Arabidopsis thaliana)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)

Evolution for FBP1 Gene

ENSEMBL:
Gene Tree for FBP1 (if available)
TreeFam:
Gene Tree for FBP1 (if available)

Paralogs for FBP1 Gene

Paralogs for FBP1 Gene

(1) SIMAP similar genes for FBP1 Gene using alignment to 3 proteins:

genes like me logo Genes that share paralogs with FBP1: view

Variants for FBP1 Gene

Sequence variations from dbSNP and Humsavar for FBP1 Gene

SNP ID Clin Chr 09 pos Sequence Context AA Info Type
VAR_002380 Fructose-1,6-bisphosphatase deficiency (FBP1D)
VAR_002381 Fructose-1,6-bisphosphatase deficiency (FBP1D)
VAR_002382 -
rs1769259 - 94,606,867(-) CGCCA(A/G)GGACT reference, missense
rs2297085 - 94,606,823(+) GGGGA(A/C/G/T)CTTCT reference, missense

Structural Variations from Database of Genomic Variants (DGV) for FBP1 Gene

Variant ID Type Subtype PubMed ID
nsv893596 CNV Gain 21882294
nsv466436 CNV Loss 19166990
esv2668617 CNV Deletion 23128226
nsv893597 CNV Gain 21882294
esv269321 CNV Insertion 20981092
esv274676 CNV Insertion 20981092
nsv415436 CNV Loss 16902084
esv2738800 CNV Deletion 23290073

Variation tolerance for FBP1 Gene

Residual Variation Intolerance Score: 50.4% of all genes are more intolerant (likely to be disease-causing)
Gene Damage Index Score: 2.61; 45.13% of all genes are more intolerant (likely to be disease-causing)

Relevant External Links for FBP1 Gene

HapMap Linkage Disequilibrium report
FBP1
Human Gene Mutation Database (HGMD)
FBP1

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for FBP1 Gene

Disorders for FBP1 Gene

MalaCards: The human disease database

(7) MalaCards diseases for FBP1 Gene - From: OMIM, ClinVar, GeneTests, Orphanet, Swiss-Prot, DISEASES, and GeneCards

Disorder Aliases PubMed IDs
fructose-1,6-bisphosphatase deficiency
  • fructose 1 phosphate aldolase deficiency
metabolic acidosis
hypoglycemia
  • hypoglycaemia
acute pyelonephritis
  • acute pyelonephritis nos
reticulate acropigmentation of kitamura
  • rapk
- elite association - COSMIC cancer census association via MalaCards
Search FBP1 in MalaCards View complete list of genes associated with diseases

UniProtKB/Swiss-Prot

F16P1_HUMAN
  • Fructose-1,6-bisphosphatase deficiency (FBP1D) [MIM:229700]: An autosomal recessive metabolic disorder characterized by impaired gluconeogenesis, and episodes of hypoglycemia and metabolic acidosis that can be lethal in newborn infants or young children. {ECO:0000269 PubMed:12126934, ECO:0000269 PubMed:25601412, ECO:0000269 PubMed:9382095}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Genatlas disease for FBP1 Gene

episodic spells of hyperventilation,apnea,hypoglycemia,metabolic acidosis and lactic acidosis with a risk of lethal course in the newborn

Relevant External Links for FBP1

Genetic Association Database (GAD)
FBP1
Human Genome Epidemiology (HuGE) Navigator
FBP1
Atlas of Genetics and Cytogenetics in Oncology and Haematology:
FBP1
genes like me logo Genes that share disorders with FBP1: view

Publications for FBP1 Gene

  1. Isolation of a human liver fructose-1,6-bisphosphatase cDNA and expression of the protein in Escherichia coli. Role of Asp-118 and Asp-121 in catalysis. (PMID: 8387495) El-Maghrabi M.R. … Pilkis S.J. (J. Biol. Chem. 1993) 2 3 4 67
  2. Warburg effect revisited: an epigenetic link between glycolysis and gastric carcinogenesis. (PMID: 19881551) Liu X. … Jin H.C. (Oncogene 2010) 3 23
  3. Novel FBP1 gene mutations in Arab patients with fructose-1,6-bisphosphatase deficiency. (PMID: 19259699) Faiyaz-Ul-Haque M. … Zaidi S.H. (Eur. J. Pediatr. 2009) 3 23
  4. Stabilizing the urinary activity of fructose-1,6-bisphosphatase with EDTA and mercaptoethanol. (PMID: 19505453) Kepka A. … Zwierz K. (Clin. Biochem. 2009) 3 23
  5. Allosteric FBPase inhibitors gain 10(5) times in potency when simultaneously binding two neighboring AMP sites. (PMID: 18650089) Hebeisen P. … Ruf A. (Bioorg. Med. Chem. Lett. 2008) 3 25

Products for FBP1 Gene

Sources for FBP1 Gene

Back to Top

Content