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LIPH Hydrolyzes specifically phosphatidic acid (PA) to produce 2-acyl lysophosphatidic acid (LPA; a potent bioactive lipid mediator) and fatty acid. Does not hydrolyze other phospholipids, like phosphatidylserine (PS), phosphatidylcholine (PC) and phosphatidylethanolamine (PE) or triacylglycerol (TG). Defects in LIPH are the cause of hypotrichosis type 7 (HYPT7); also known as alopecia universalis congenita Mari type. A condition characterized by the presence of less than the normal amount of hair. Affected individuals have sparse or absent scalp, axillary and body hair and sparse eyebrows and eyelashes. Defects in LIPH are the cause of woolly hair autosomal recessive type 2 (ARWH2). A hair shaft disorder characterized by fine and tightly curled hair. Compared to normal curly hair that is observed in some populations, woolly hair grows slowly and stops growing after a few inches. Under light microscopy, woolly hair shows some structural anomalies, including trichorrhexis nodosa and tapered ends. Some individuals may present with hypotrichosis. Belongs to the AB hydrolase superfamily. Lipase family. Note: This description may include information from UniProtKB.
Protein type: EC 3.1.1.-; Phospholipase; Secreted; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 3q27.2
Cellular Component: extracellular space; plasma membrane
Molecular Function: heparin binding; phospholipase activity
Biological Process: lipid catabolic process; phosphatidic acid biosynthetic process
Disease: Hypotrichosis 7
Reference #:  Q8WWY8 (UniProtKB)
Alt. Names/Synonyms: AH; lipase H; Lipase member H; lipase, member H; LIPH; LPD lipase-related protein; LPDLR; Membrane-associated phosphatidic acid-selective phospholipase A1-alpha; membrane-bound phosphatidic acid-selective phospholipase A1; mPA-PLA1; mPA-PLA1 alpha; MPAPLA1; Phospholipase A1 member B; PLA1B
Gene Symbols: LIPH
Molecular weight: 50,859 Da
Basal Isoelectric point: 7.15  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

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