Family

Phosphatidate phosphatase LPIN1 (IPR028794)

Short name: LPIN1

Family relationships

None.

Description

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].

GO terms

Biological Process

GO:0009062 fatty acid catabolic process
GO:0006357 regulation of transcription from RNA polymerase II promoter
GO:0019432 triglyceride biosynthetic process

Molecular Function

GO:0008195 phosphatidate phosphatase activity

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.
PANTHER