Phosphatidate phosphatase LPIN1 (IPR028794)
Short name: LPIN1
Overlapping homologous superfamilies
- LIPIN family (IPR026058)
- Phosphatidate phosphatase LPIN1 (IPR028794)
Phosphatidate phosphatase Lpin1 (or Lipin-1) belongs to the Lipin family, which consists of magnesium-dependent phosphatidate phosphatase enzymes that provide diacylglycerol for synthesis of triacylglycerol and phospholipids [PMID: 20645851]. Besides the triglyceride synthesis function, Lpin1 also regulates gene expression through direct protein-protein interactions with DNA-bound transcription factors in liver [PMID: 23236470]. Its interaction with the hepatocyte nuclear factor 4alpha (HNF4alpha) affects liver lipid metabolism [PMID: 23236470]. Lpin1 can also activate mitochondrial fatty acid oxidative metabolism through the hepatic PGC-1alpha/PPARalpha regulatory pathway [PMID: 16950137].
Defects in Lpin1 are the cause of the fatty liver dystrophy phenotype (fld) in mice [PMID: 11138012]. Mutations in Lpin1 cause human myoglobinuria, acute recurrent, autosomal recessive (ARARM), which is a recurrent myoglobinuria characteried by recurrent attacks of rhabdomyolysis associated with muscle pain and weakness and followed by excretion of myoglobin in the urine [PMID: 18817903].
- PTHR12181:SF10 (PTHR12181:SF10)