Summary: Fructose-1-6-bisphosphatase, N-terminal domain
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Fructose 1,6-bisphosphatase Edit Wikipedia article
Fructose bisphosphatase is an enzyme in the liver, that converts fructose-1,6-bisphosphate to fructose-6-phosphate in gluconeogenesis (the making of glucose from smaller substrates). Fructose bisphosphatase does the opposite job to phosphofructokinase, and both these enzymes only work in one direction.
Fructose bisphosphatase deficiency
If there is a deficiency in fructose bisphosphatase, gluconeogenesis will not occur correctly. Glycolysis (the break-down of glucose) will still work, as this does not use this enzyme.
Without effective gluconeogenesis (GNG), hypoglycaemia will set in after about 12 hours. This is the time when liver glycogen stores have been exhausted, and the body has to rely on GNG. When given a dose of glucagon (which would normally increase blood glucose) nothing will happen, as stores are depleted and GNG doesn't work. (In fact, the patient would already have high glucagon levels.)
There is no problem with the metabolism of glucose or galactose, but fructose and glycerol cannot be used as fuels. If fructose or glycerol are given, there will be a build up of phophorylated three-carbon sugars. This leads to phosphate depletion within the cells, and also in the blood. Without phosphate, ATP cannot be made, and many cell processes cannot occur.
High levels of glucagon will tend to release fatty acids from adipose tissue, and this will combine with glycerol that cannot be used in the liver, to make triacylglycerides causing a fatty liver.
As three carbon molecules cannot be used to make glucose, the will instead be made into pyruvate and lactate. These acids cause a drop in the pH of the blood (a metabolic acidosis). Acetyl CoA will also build up, leading to the creation of ketone bodies.
To treat people with a deficiency of this enzyme, they must avoid needing gluconeogenesis to make glucose. This can be accomplished by not fasting for long periods, and eating high-carbohydrate food. They should avoid fructose containing foods (as well as sucrose which breaks down to fructose).
As with all single-gene metabolic disorders, there is always hope for genetic therapy, inserting a healthy copy of the gene into the liver.
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Fructose-1-6-bisphosphatase, N-terminal domain Provide feedback
This family represents the N-terminus of this protein family.
Weeks CM, Roszak AW, Erman M, Kaiser R, Jornvall H, Ghosh D; , Acta Crystallogr D Biol Crystallogr 1999;55:93-102.: Structure of rabbit liver fructose 1,6-bisphosphatase at 2.3 A resolution. PUBMED:10089399 EPMC:10089399
Internal database links
|Similarity to PfamA using HHSearch:||Inositol_P|
External database links
This tab holds annotation information from the InterPro database.
InterPro entry IPR033391
Fructose-1,6-bisphosphatase (FBPase) is a critical regulatory enzyme in gluconeogenesis that catalyses the removal of 1-phosphate from fructose 1,6-bis-phosphate to form fructose 6-phosphate [ PUBMED:2159755 , PUBMED:3008716 ]. Five different classes (or types) of FBPases have been identified based on their amino acid sequences, with class I most widely distributed among living organisms [ PUBMED:16670087 ].
This entry represents the N terminus of the FBPase class 1 family.
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This family is a member of clan Phospoesterase (CL0171), which has the following description:
Members of this clan show metal-dependent / lithium sensitive phosphomonoesterase activity. The clan includes inositol polyphosphate 1 phosphatase and fructose 1,6-bisphosphatase .
The clan contains the following 3 members:FBPase FBPase_glpX Inositol_P
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1Cannot generate PP/Heatmap alignments for seeds; no PP data available
Key: available, not generated, — not available.
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|Author:||Finn RD , Griffiths-Jones SR|
|Number in seed:||7|
|Number in full:||6892|
|Average length of the domain:||179.3 aa|
|Average identity of full alignment:||40 %|
|Average coverage of the sequence by the domain:||52.83 %|
|HMM build commands:||
build method: hmmbuild -o /dev/null HMM SEED
search method: hmmsearch -Z 61295632 -E 1000 --cpu 4 HMM pfamseq
|Family (HMM) version:||23|
|Download:||download the raw HMM for this family|
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For those sequences which have a structure in the Protein DataBank, we use the mapping between UniProt, PDB and Pfam coordinate systems from the PDBe group, to allow us to map Pfam domains onto UniProt sequences and three-dimensional protein structures. The table below shows the structures on which the FBPase domain has been found. There are 442 instances of this domain found in the PDB. Note that there may be multiple copies of the domain in a single PDB structure, since many structures contain multiple copies of the same protein sequence.
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AlphaFold Structure Predictions
The list of proteins below match this family and have AlphaFold predicted structures. Click on the protein accession to view the predicted structure.