CERT1
Domain
The START domain recognizes ceramides and diacylglycerol lipids, interacts with membranes, and mediates the intermembrane transfer of ceramides and diacylglycerol lipids.
The PH domain targets the Golgi apparatus.
The FFAT motif is required for interaction with VAPA, VAPB and MOSPD2.
Function
Shelters ceramides and diacylglycerol lipids inside its START domain and mediates the intracellular trafficking of ceramides and diacylglycerol lipids in a non-vesicular manner.
Involvement in disease
Intellectual developmental disorder, autosomal dominant 34
MRD34
A disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptive behavior and manifested during the developmental period.
None
The disease is caused by variants affecting the gene represented in this entry.
Post-translational modifications
Phosphorylation on Ser-132 decreases the affinity toward phosphatidylinositol 4-phosphate at Golgi membranes and reduces ceramide transfer activity. Inactivated by hyperphosphorylation of serine residues by CSNK1G2/CK1 that triggers dissociation from the Golgi complex, thus down-regulating ER-to-Golgi transport of ceramide and sphingomyelin synthesis.
Tissue Specificity
Widely expressed.
Cellular localization
- Cytoplasm
- Golgi apparatus
- Endoplasmic reticulum
- Preferentially localized to the Golgi apparatus.
Alternative names
CERT, COL4A3BP, STARD11, CERT1, Ceramide transfer protein, hCERT, Collagen type IV alpha-3-binding protein, Goodpasture antigen-binding protein, START domain-containing protein 11, StAR-related lipid transfer protein 11, GPBP, StARD11