FBP1
entrez
2203
canonical
uniprot
P09467
canonical
ensembl
ENSG00000165140
resolver
ensp
ENSP00000364475
resolver
ensp
ENSP00000408025
resolver
ensp
ENSP00000554926
resolver
ensp
ENSP00000554928
resolver
ensp
ENSP00000554929
resolver
ensp
ENSP00000554930
resolver
enst
ENST00000375326
resolver
enst
ENST00000415431
resolver
enst
ENST00000884867
resolver
enst
ENST00000884869
resolver
enst
ENST00000884870
resolver
enst
ENST00000884871
resolver
genesymbol
FBP1
resolver
genesymbol_syn
FBP
resolver
hgnc
HGNC:3606
resolver
uniprot
O75571
resolver
uniprot
Q2TU34
resolver
uniprot
Q53F94
resolver
uniprot
Q96E46
resolver
uniprot_entry
F16P1_HUMAN
resolver
uniprot_entry
Q2TU34_HUMAN
resolver
uniprot_sec
O75571
resolver
uniprot_sec
Q53F94
resolver
uniprot_sec
Q96E46
resolver
activity_regulation
ACTIVITY REGULATION: 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 an allosteric inhibitor. AMP binding affects the turnover of bound substrate and not the affinity for substrate. Fructose 2,6-bisphosphate acts as a competitive inhibitor. Fructose 2,6-bisphosphate and AMP have synergistic effects. {ECO:0000269|PubMed:16442285, ECO:0000269|PubMed:16446092, ECO:0000269|PubMed:17350621, ECO:0000269|PubMed:18650089, ECO:0000269|PubMed:7809062}.
uniprot
proteins
amino_acid_sequence
MADQAPFDTDVNTLTRFVMEEGRKARGTGELTQLLNSLCTAVKAISSAVRKAGIAHLYGIAGSTNVTGDQVKKLDVLSNDLVMNMLKSSFATCVLVSEEDKHAIIVEPEKRGKYVVCFDPLDGSSNIDCLVSVGTIFGIYRKKSTDEPSEKDALQPGRNLVAAGYALYGSATMLVLAMDCGVNCFMLDPAIGEFILVDKDVKIKKKGKIYSLNEGYARDFDPAVTEYIQRKKFPPDNSAPYGARYVGSMVADVHRTLVYGGIFLYPANKKSPNGKLRLLYECNPMAYVMEKAGGMATTGKEAVLDVIPTDIHQRAPVILGSPDDVLEFLKVYEKHSAQ
uniprot
proteins
disease_involvement
DISEASE: 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 variants affecting the gene represented in this entry.
uniprot
proteins
ec_number
3.1.3.11
uniprot
proteins
function
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. {ECO:0000269|PubMed:16497803, ECO:0000269|PubMed:18375435, ECO:0000269|PubMed:22517657}.
uniprot
proteins
mass_dalton
36842
uniprot
proteins
mutagenesis
MUTAGEN 70; /note="Q->E: Increased affinity towards Ca(2+) and Mg(2+)."; /evidence="ECO:0000269|PubMed:17350621"; MUTAGEN 119; /note="D->A: Reduced activity."; /evidence="ECO:0000269|PubMed:8387495"; MUTAGEN 122; /note="D->A: Reduced activity."; /evidence="ECO:0000269|PubMed:8387495
uniprot
proteins
pathway_participation
PATHWAY: Carbohydrate biosynthesis; gluconeogenesis.
uniprot
proteins
protein_family
FBPase class 1 family
uniprot
proteins
pubmed
10222032
uniprot
proteins
pubmed
12126934
uniprot
proteins
pubmed
15164053
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16442285
uniprot
proteins
pubmed
16446092
uniprot
proteins
pubmed
16497803
uniprot
proteins
pubmed
17350621
uniprot
proteins
pubmed
18375435
uniprot
proteins
pubmed
18650089
uniprot
proteins
pubmed
22517657
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
25601412
uniprot
proteins
pubmed
2842796
uniprot
proteins
pubmed
7558035
uniprot
proteins
pubmed
7589895
uniprot
proteins
pubmed
7809062
uniprot
proteins
pubmed
8135811
uniprot
proteins
pubmed
8382525
uniprot
proteins
pubmed
8387495
uniprot
proteins
pubmed
9382095
uniprot
proteins
sequence_length
338
uniprot
proteins