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PNLIP

Function

Plays an important role in fat metabolism. It preferentially splits the esters of long-chain fatty acids at positions 1 and 3, producing mainly 2-monoacylglycerol and free fatty acids, and shows considerably higher activity against insoluble emulsified substrates than against soluble ones.

Involvement in disease

Pancreatic lipase deficiency

PNLIPD

An autosomal recessive disorder characterized by exocrine pancreatic failure. Clinical findings include oily/greasy stools from infancy or early childhood, absence of discernible pancreatic disease, and significantly decreased pancreatic lipolytic activity.

None

The disease is caused by variants affecting the gene represented in this entry.

Sequence Similarities

Belongs to the AB hydrolase superfamily. Lipase family.

Cellular localization

Alternative names

Pancreatic triacylglycerol lipase, PL, PTL, Pancreatic lipase, PNLIP

swissprot:P16233 omim:246600 entrezGene:5406