PNLIP
entrez
5406
canonical
uniprot
P16233
canonical
ensembl
ENSG00000175535
resolver
ensp
ENSP00000358223
resolver
enst
ENST00000369221
resolver
genesymbol
PNLIP
resolver
hgnc
HGNC:9155
resolver
uniprot
Q5VSQ2
resolver
uniprot_entry
LIPP_HUMAN
resolver
uniprot_sec
Q5VSQ2
resolver
activity_regulation
ACTIVITY REGULATION: Inhibited by bile salts, is reactivated by (pro)colipase/CLPS. {ECO:0000269|PubMed:10769148}.
uniprot
proteins
amino_acid_sequence
MLPLWTLSLLLGAVAGKEVCYERLGCFSDDSPWSGITERPLHILPWSPKDVNTRFLLYTNENPNNFQEVAADSSSISGSNFKTNRKTRFIIHGFIDKGEENWLANVCKNLFKVESVNCICVDWKGGSRTGYTQASQNIRIVGAEVAYFVEFLQSAFGYSPSNVHVIGHSLGAHAAGEAGRRTNGTIGRITGLDPAEPCFQGTPELVRLDPSDAKFVDVIHTDGAPIVPNLGFGMSQVVGHLDFFPNGGVEMPGCKKNILSQIVDIDGIWEGTRDFAACNHLRSYKYYTDSIVNPDGFAGFPCASYNVFTANKCFPCPSGGCPQMGHYADRYPGKTNDVGQKFYLDTGDASNFARWRYKVSVTLSGKKVTGHILVSLFGNKGNSKQYEIFKGTLKPDSTHSNEFDSDVDVGDLQMVKFIWYNNVINPTLPRVGASKIIVETNVGKQFNFCSPETVREEVLLTLTPC
uniprot
proteins
disease_involvement
DISEASE: Pancreatic lipase deficiency (PNLIPD) [MIM:614338]: 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. {ECO:0000269|PubMed:24262094, ECO:0000269|PubMed:25862608}. Note=The disease is caused by variants affecting the gene represented in this entry.
uniprot
proteins
ec_number
3.1.1.3
uniprot
proteins
function
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. {ECO:0000269|PubMed:10769148, ECO:0000269|PubMed:17401110}.
uniprot
proteins
mass_dalton
51157
uniprot
proteins
protein_family
AB hydrolase superfamily
uniprot
proteins
protein_family
Lipase family
uniprot
proteins
pubmed
10769148
uniprot
proteins
pubmed
1379598
uniprot
proteins
pubmed
14702039
uniprot
proteins
pubmed
15164054
uniprot
proteins
pubmed
1522902
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
17401110
uniprot
proteins
pubmed
2106079
uniprot
proteins
pubmed
24262094
uniprot
proteins
pubmed
2479644
uniprot
proteins
pubmed
25862608
uniprot
proteins
pubmed
26494624
uniprot
proteins
pubmed
7893686
uniprot
proteins
pubmed
8029213
uniprot
proteins
pubmed
8406023
uniprot
proteins
pubmed
8479519
uniprot
proteins
sequence_length
465
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Secreted {ECO:0000269|PubMed:25862608}.
uniprot
proteins