Gene Details: FPGT


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General Information

Gene Name: FPGT (Fucose-1-phosphate guanylyltransferase)
Synonym: GFPP
Short Names:
Alternative Names: GDP-L-fucose diphosphorylase;GDP-L-fucose pyrophosphorylase;

Notes:


Description from Dr.Glyco-GPT:

Write a 100-350 word essay about the enzyme human FPGT. Provide information related to enzyme: i) function, ii) reaction pathways, ii) Location, and iv) diseases. In writing include these four subheadings

### Function

Human FPGT (fucose-1-phosphate guanylyltransferase) is an enzyme that plays a key role in the salvage pathway of GDP-fucose synthesis. FPGT catalyzes the conversion of fucose-1-phosphate into GDP-fucose, an essential donor molecule for the fucosylation of glycoproteins and glycolipids. Fucosylation is critical for various biological processes, including cell adhesion, immune response, and signal transduction .

### Reaction Pathways

FPGT is involved in the fucose salvage pathway, a process that allows cells to recycle fucose released from the degradation of glycoconjugates. The enzyme catalyzes the formation of GDP-fucose from fucose-1-phosphate and GTP. This reaction is vital for maintaining adequate levels of GDP-fucose, particularly in cells with high demands for fucosylation. The GDP-fucose produced is used by fucosyltransferases in the Golgi apparatus to modify glycoproteins and glycolipids  .

### Location

FPGT is primarily localized in the cytoplasm, where it performs its role in the fucose salvage pathway. This cytoplasmic localization enables FPGT to efficiently interact with its substrates, ensuring a steady supply of GDP-fucose for fucosylation processes. After synthesis, GDP-fucose is transported to the Golgi apparatus, where it is used for glycan modification .

### Diseases

While no direct human diseases have been conclusively linked to FPGT mutations, its function is critical for proper fucosylation. Defects in fucosylation are associated with various disorders, including leukocyte adhesion deficiency type II (LAD II), a condition characterized by immune deficiencies and developmental abnormalities. Additionally, FPGT has been implicated in viral myocarditis, where genetic variants may influence susceptibility to the disease .

In summary, FPGT is an essential enzyme in the GDP-fucose biosynthesis pathway, ensuring proper fucosylation of glycoproteins and glycolipids. Its cytoplasmic activity is crucial for maintaining cellular functions, and disruptions in this pathway could have significant implications for human health.

Catalytic Activity

Reaction Image

Reaction and Disease Links

EC # (IUBMB): 2.7.7.30
Brenda: 2.7.7.30
OMIM: 603609
KEGG: 8790
Rhea: 13549
Reactome : R-HSA-6787639

Transcript levels (Cell lines and Single cell data) URL


CRISPR-knockout

CRISPR-activation

CRISPR-inactivation


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