PCSK1N
entrez
27344
canonical
uniprot
Q9UHG2
canonical
ensembl
ENSG00000102109
resolver
ensp
ENSP00000218230
resolver
enst
ENST00000218230
resolver
genesymbol
PCSK1N
resolver
hgnc
HGNC:17301
resolver
uniprot
Q4VC04
resolver
uniprot_entry
PCS1N_HUMAN
resolver
uniprot_sec
Q4VC04
resolver
amino_acid_sequence
MAGSPLLWGPRAGGVGLLVLLLLGLFRPPPALCARPVKEPRGLSAASPPLAETGAPRRFRRSVPRGEAAGAVQELARALAHLLEAERQERARAEAQEAEDQQARVLAQLLRVWGAPRNSDPALGLDDDPDAPAAQLARALLRARLDPAALAAQLVPAPVPAAALRPRPPVYDDGPAGPDAEEAGDETPDVDPELLRYLLGRILAGSADSEGVAAPRRLRRAADHDVGSELPPEGVLGALLRVKRLETPAPQVPARRLLPP
uniprot
proteins
function
FUNCTION: May act as a protein chaperone that prevents the aggregation of client proteins and plays a role in neuroprotection (PubMed:24042052). Blocks the trans-synaptic spread of SNCA/alpha-synuclein in the brain. May form spheres that encapsulate client proteins (By similarity). May prevent aggregation of amyloid beta, IAPP/islet amyloid polypeptide, and SNCA/alpha-synuclein (PubMed:24042052). Cannot disaggregate preformed fibrils. Additionally, may function in the control of the neuroendocrine secretory pathway by acting as a specific endogenous inhibitor of PCSK1. ProSAAS and Big PEN-LEN, both containing the C-terminal inhibitory domain, reduce the activity of the 87 kDa form of PCSK1, but not the autocatalytically derived 66 kDa form, in the endoplasmic reticulum and Golgi. May control the intracellular timing of PCSK1 rather than its total level of activity and slows down convertase-mediated processing of proopiomelanocortin and proenkephalin (By similarity). {ECO:0000250|UniProtKB:Q9QXV0, ECO:0000269|PubMed:24042052}.; FUNCTION: [Big LEN]: Endogenous ligand for GPR171. Neuropeptide involved in the regulation of feeding. {ECO:0000250|UniProtKB:Q9QXV0}.; FUNCTION: [PEN]: Endogenous ligand for GPR83. Neuropeptide involved in the regulation of feeding. {ECO:0000250|UniProtKB:Q9QXV0}.
uniprot
proteins
mass_dalton
27372
uniprot
proteins
mutagenesis
MUTAGEN 235; /note="V->A: Reduces inhibition of PCSK1."; /evidence="ECO:0000269|PubMed:11435430"; MUTAGEN 236; /note="L->A: Greatly reduces inhibition of PCSK1."; /evidence="ECO:0000269|PubMed:11435430"; MUTAGEN 237; /note="G->A: Reduces inhibition of PCSK1."; /evidence="ECO:0000269|PubMed:11435430"; MUTAGEN 240; /note="L->A: Reduces inhibition of PCSK1."; /evidence="ECO:0000269|PubMed:11435430"; MUTAGEN 241; /note="R->A: Reduces inhibition of PCSK1."; /evidence="ECO:0000269|PubMed:11435430"; MUTAGEN 242; /note="V->A: Reduces inhibition of PCSK1."; /evidence="ECO:0000269|PubMed:11435430"; MUTAGEN 243; /note="K->A: Abolishes inhibition of PCSK1."; /evidence="ECO:0000269|PubMed:11435430"; MUTAGEN 244; /note="R->A: Abolishes inhibition of PCSK1."; /evidence="ECO:0000269|PubMed:11435430"; MUTAGEN 245; /note="L->A: Reduces inhibition of PCSK1."; /evidence="ECO:0000269|PubMed:11435430"; MUTAGEN 246; /note="E->A: Reduces inhibition of PCSK1."; /evidence="ECO:0000269|PubMed:11435430
uniprot
proteins
post_translational_modification
PTM: Proteolytically cleaved in the Golgi. {ECO:0000269|PubMed:12914799}.; PTM: O-glycosylated with a core 1 or possibly core 8 glycan. {ECO:0000269|PubMed:19838169, ECO:0000269|PubMed:22171320, ECO:0000269|PubMed:23234360}.
uniprot
proteins
pubmed
10632593
uniprot
proteins
pubmed
11435430
uniprot
proteins
pubmed
12914799
uniprot
proteins
pubmed
14746899
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
19838169
uniprot
proteins
pubmed
22171320
uniprot
proteins
pubmed
23234360
uniprot
proteins
pubmed
24042052
uniprot
proteins
pubmed
24102330
uniprot
proteins
pubmed
27457957
uniprot
proteins
sequence_length
260
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Secreted {ECO:0000269|PubMed:24102330}. Golgi apparatus, trans-Golgi network {ECO:0000250|UniProtKB:Q9QXV0}. Note=Proteotoxic stress stimulates its intracellular retention (By similarity). An N-terminal processed peptide, probably Big SAAS or Little SAAS, is accumulated in cytoplasmic protein tau deposits in frontotemporal dementia and parkinsonism linked to chromosome 17 (Pick disease), Alzheimer disease and amyotrophic lateral sclerosis-parkinsonism/dementia complex 1 (Guam disease) (PubMed:12914799, PubMed:14746899). Colocalizes with extracellular amyloid plaques in Alzheimer disease (PubMed:24102330). {ECO:0000250|UniProtKB:Q9QXV0, ECO:0000269|PubMed:12914799, ECO:0000269|PubMed:14746899, ECO:0000269|PubMed:24102330}.
uniprot
proteins