LPGAT1
Description
The LPGAT1 (lysophosphatidylglycerol acyltransferase 1) is a protein-coding gene located on chromosome 1.
LPGAT1 is a lysophospholipid acyltransferase involved in fatty acyl chain remodeling of glycerophospholipids in the endoplasmic reticulum membrane. It specifically catalyzes the transfer and esterification of saturated long-chain fatty acids from acyl-CoA to the sn-1 position of 1-lyso-2-acyl phosphatidylethanolamines (1-lyso-PE, LPE), favoring stearoyl CoA over palmitoyl CoA as the acyl donor. This activity, in conjunction with an unidentified phospholipase A1, facilitates the conversion of palmitate phosphatidylethanolamine (PE) species into stearate ones. LPGAT1 provides substrates for the PE methylation pathway, thereby controlling the stearate/palmitate composition of PE and phosphatidylcholine (PC) species. This ultimately impacts de novo hepatic lipid synthesis, body fat content, and lifespan. Additionally, LPGAT1 can acylate lysophosphatidylglycerols (LPG) using various saturated fatty acyl-CoAs as acyl donors. It also exhibits acyltransferase activity towards monoacylglycerols, with a preference for 2-monoacylglycerols over 1-monoacylglycerols. However, LPGAT1 shows no activity towards lysophosphatidic acids (LPA).
LPGAT1 is also known as FAM34A, FAM34A1, LPLAT7, NET8.
Associated Diseases
- Gorham-Stout disease
- melorheostosis
- X-linked retinal dysplasia
- severe early-childhood-onset retinal dystrophy
- X-linked retinoschisis
- birdshot chorioretinopathy
- exudative vitreoretinopathy 2, X-linked
- retinitis pigmentosa
