FBP1

Protein identityUniProt · HPA

UniProt accession

P09467

Gene

FBP1

Ensembl

ENSG00000165140

Chromosome

9

Canonical length

338 aa

Protein class

Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Plasma proteins, Potential drug targets, Predicted intracellular proteins

Subcellular location

Mitochondria

Quaternary structure

Homotetramer

OverviewNCBI 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]

Canonical amino-acid sequenceUniProt

338 residues, UniProt reviewed canonical sequence.

>P09467|FBP1
     1  MADQAPFDTD VNTLTRFVME EGRKARGTGE LTQLLNSLCT AVKAISSAVR KAGIAHLYGI
    61  AGSTNVTGDQ VKKLDVLSND LVMNMLKSSF ATCVLVSEED KHAIIVEPEK RGKYVVCFDP
   121  LDGSSNIDCL VSVGTIFGIY RKKSTDEPSE KDALQPGRNL VAAGYALYGS ATMLVLAMDC
   181  GVNCFMLDPA IGEFILVDKD VKIKKKGKIY SLNEGYARDF DPAVTEYIQR KKFPPDNSAP
   241  YGARYVGSMV ADVHRTLVYG GIFLYPANKK SPNGKLRLLY ECNPMAYVME KAGGMATTGK
   301  EAVLDVIPTD IHQRAPVILG SPDDVLEFLK VYEKHSAQ

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against FBP1 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability

Intracellular

Secreted

No

Transmembrane segments

0

Mean surface accessibility (rSASA)

0.24

Highest tissue expression

819 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about FBP1.

Disease | AllUniProt

Conditions FBP1 is implicated in, by any mechanism.

• Fructose-1,6-bisphosphatase deficiency (FBP1D) MIM:229700

Disease | GeneticClinVar

58 pathogenic / likely-pathogenic of 349 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Fructose-biphosphatase deficiency
• FBP1-related disorder
• Inborn genetic diseases
• See cases
• Deficiency of fructose-bisphosphatase

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)

0.72

gnomAD pLI

0.08

gnomAD missense Z

0.74

DepMap mean gene effect

-0.1

DepMap dependency class

selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 3% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

• cellular hyperosmotic salinity response
• cellular hypotonic salinity response
• cellular response to cAMP
• cellular response to insulin stimulus
• cellular response to magnesium ion
• cellular response to phorbol 13-acetate 12-myristate
• cellular response to raffinose
• cellular response to xenobiotic stimulus
• fructose 1,6-bisphosphate metabolic process
• fructose 6-phosphate metabolic process
• fructose metabolic process
• gluconeogenesis
• negative regulation of cell growth
• negative regulation of glycolytic process
• negative regulation of Ras protein signal transduction
• negative regulation of transcription by RNA polymerase II
• regulation of gluconeogenesis
• response to nutrient levels

Molecular functions

• AMP binding
• fructose 1,6-bisphosphate 1-phosphatase activity
• identical protein binding
• metal ion binding
• monosaccharide binding
• RNA polymerase II-specific DNA-binding transcription factor binding

Cellular components

• cytoplasm
• cytosol
• extracellular exosome
• nucleus

Protein domainsUniProt · Pfam · InterPro

• Fructose-1,6-bisphosphatase class 1
• Fructose-1,6-bisphosphatase, active site
• Fructose-1,6-bisphosphatase
• Fructose-1-6-bisphosphatase class I, N-terminal
• Fructose-1-6-bisphosphatase class 1, C-terminal
• Fructose-1-6-bisphosphatase, N-terminal domain
• Fructose-1-6-bisphosphatase, C-terminal domain

KeywordsUniProt

• Acetylation
• Allosteric enzyme
• Carbohydrate metabolism
• Gluconeogenesis
• Hydrolase
• Magnesium
• Metal-binding
• Phosphoprotein

InteractionsUniProt · HPA

Protein binding partners of FBP1 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• BIN1
• FBP2