ORMDL2
Description
The ORMDL2 (ORMDL sphingolipid biosynthesis regulator 2) is a protein-coding gene located on chromosome 12.
ORMDL2 plays a key role in maintaining the balance of sphingolipid production by regulating the activity of the serine palmitoyltransferase (SPT) enzyme. It responds to changes in ceramide levels, a type of sphingolipid. When ORMDL2 binds to SPT, ceramides attach to its N-terminal region, causing a structural change that blocks the entry of SPT substrates, thus inhibiting the enzyme's activity. This mechanism ensures sufficient ceramide levels for complex sphingolipid synthesis while preventing excessive accumulation of ceramides, which can induce cell death.
ORMDL2 is also known as HSPC160, MST095, MSTP095, adoplin-2.
