ORMDL1
Description
The ORMDL1 (ORMDL sphingolipid biosynthesis regulator 1) is a protein-coding gene located on chromosome 2.
ORM1-like protein 1, also known as Adoplin-1, is a protein that in humans is encoded by the ORMDL1 gene. It plays an essential role in the homeostatic regulation of sphingolipid de novo biosynthesis by modulating the activity of the serine palmitoyltransferase (SPT) in response to ceramide levels. When complexed to SPT, the binding of ceramides to its N-terminus stabilizes a conformation that blocks SPT substrate entry, hence preventing SPT catalytic activity. Through this mechanism, maintains ceramide levels at sufficient concentrations for the production of complex sphingolipids, but which prevents the accumulation of ceramides to levels that trigger apoptosis (By similarity). Ceramide-sensitive subunit of the serine palmitoyltransferase (SPT) complex, which is also composed of SPTLC1, SPTLC2/3 and SPTSSA/B.
ORM1-like protein 1 plays a crucial role in controlling the production of sphingolipids, a type of fat molecule. It does this by adjusting the activity of an enzyme called serine palmitoyltransferase (SPT) in response to changes in ceramide levels. When ORMDL1 binds to SPT, ceramides attach to its N-terminus, causing a change in SPT's shape that blocks the entry of substrates. This stops SPT from working, preventing the accumulation of ceramides to harmful levels that could trigger cell death. This mechanism ensures that there are enough ceramides to create complex sphingolipids, while also preventing an excessive buildup of ceramides.
ORMDL1 is also known as -.
