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Protein

Serine protease inhibitor Kazal-type 1

Gene

SPINK1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Serine protease inhibitor which exhibits anti-trypsin activity (PubMed:7142173). In the pancreas, protects against trypsin-catalyzed premature activation of zymogens (By similarity).By similarity1 Publication
In the male reproductive tract, binds to sperm heads where it modulates sperm capacitance by inhibiting calcium uptake and nitrogen oxide (NO) production.By similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sitei41 – 42Reactive bond for trypsinPROSITE-ProRule annotationBy similarity2
Sitei43 – 44Necessary for sperm bindingBy similarity2

GO - Molecular functioni

  • endopeptidase inhibitor activity Source: ProtInc
  • serine-type endopeptidase inhibitor activity Source: GO_Central

GO - Biological processi

Keywordsi

Molecular functionProtease inhibitor, Serine protease inhibitor

Protein family/group databases

MEROPSiI01.011

Names & Taxonomyi

Protein namesi
Recommended name:
Serine protease inhibitor Kazal-type 1Imported
Alternative name(s):
Pancreatic secretory trypsin inhibitor1 Publication
Tumor-associated trypsin inhibitor
Short name:
TATI
Gene namesi
Name:SPINK1
Synonyms:PSTI
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 5

Organism-specific databases

EuPathDBiHostDB:ENSG00000164266.10
HGNCiHGNC:11244 SPINK1
MIMi167790 gene
neXtProtiNX_P00995

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Secreted

Pathology & Biotechi

Involvement in diseasei

Pancreatitis, hereditary (PCTT)5 Publications
Disease susceptibility is associated with variations affecting the gene represented in this entry.
Disease descriptionA disease characterized by pancreas inflammation, permanent destruction of the pancreatic parenchyma, maldigestion, and severe abdominal pain attacks.
See also OMIM:167800
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_03201112L → F in PCTT. 1 PublicationCorresponds to variant dbSNP:rs35877720EnsemblClinVar.1
Natural variantiVAR_01168814L → P in PCTT. 1 PublicationCorresponds to variant dbSNP:rs104893939EnsemblClinVar.1
Tropical calcific pancreatitis (TCP)2 Publications
Disease susceptibility is associated with variations affecting the gene represented in this entry.
Disease descriptionIdiopathic, juvenile, nonalcoholic form of chronic pancreatitis widely prevalent in several tropical countries. It can be associated with fibrocalculous pancreatic diabetes (FCPD) depending on both environmental and genetic factors. TCP differs from alcoholic pancreatitis by a much younger age of onset, pancreatic calcification, a high incidence of insulin dependent but ketosis resistant diabetes mellitus, and an exceptionally high incidence of pancreatic cancer.
See also OMIM:608189

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi6690
MalaCardsiSPINK1
MIMi167800 phenotype
608189 phenotype
OpenTargetsiENSG00000164266
Orphaneti676 Hereditary chronic pancreatitis
103918 Tropical pancreatitis
PharmGKBiPA36074

Polymorphism and mutation databases

BioMutaiSPINK1
DMDMi124856

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 232 PublicationsAdd BLAST23
ChainiPRO_000001655724 – 79Serine protease inhibitor Kazal-type 1Add BLAST56

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Disulfide bondi32 ↔ 61
Disulfide bondi39 ↔ 58
Disulfide bondi47 ↔ 79

Keywords - PTMi

Disulfide bond

Proteomic databases

MaxQBiP00995
PaxDbiP00995
PeptideAtlasiP00995
PRIDEiP00995

PTM databases

iPTMnetiP00995
PhosphoSitePlusiP00995

Miscellaneous databases

PMAP-CutDBiP00995

Expressioni

Gene expression databases

BgeeiENSG00000164266
CleanExiHS_SPINK1
ExpressionAtlasiP00995 baseline and differential
GenevisibleiP00995 HS

Organism-specific databases

HPAiCAB026366
HPA027498

Interactioni

Protein-protein interaction databases

BioGridi112568, 3 interactors
MINTiP00995
STRINGi9606.ENSP00000296695

Structurei

Secondary structure

179
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi26 – 28Combined sources3
Beta strandi37 – 40Combined sources4
Beta strandi46 – 48Combined sources3
Beta strandi53 – 56Combined sources4
Helixi57 – 67Combined sources11
Beta strandi73 – 77Combined sources5

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1CGIX-ray2.30I24-79[»]
1CGJX-ray2.30I24-79[»]
1HPTX-ray2.30A24-79[»]
ProteinModelPortaliP00995
SMRiP00995
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP00995

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini26 – 79Kazal-likePROSITE-ProRule annotationAdd BLAST54

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiKOG3649 Eukaryota
ENOG410YC3T LUCA
GeneTreeiENSGT00530000064228
HOGENOMiHOG000090244
HOVERGENiHBG006182
InParanoidiP00995
OMAiTKIYNPV
OrthoDBiEOG091G14HG
PhylomeDBiP00995

Family and domain databases

InterProiView protein in InterPro
IPR002350 Kazal_dom
IPR036058 Kazal_dom_sf
IPR001239 Prot_inh_Kazal-m
PfamiView protein in Pfam
PF00050 Kazal_1, 1 hit
PRINTSiPR00290 KAZALINHBTR
SMARTiView protein in SMART
SM00280 KAZAL, 1 hit
SUPFAMiSSF100895 SSF100895, 1 hit
PROSITEiView protein in PROSITE
PS00282 KAZAL_1, 1 hit
PS51465 KAZAL_2, 1 hit

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

P00995-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MKVTGIFLLS ALALLSLSGN TGADSLGREA KCYNELNGCT KIYDPVCGTD
60 70
GNTYPNECVL CFENRKRQTS ILIQKSGPC
Length:79
Mass (Da):8,507
Last modified:July 1, 1989 - v2
Checksum:i3583C8196952EB3A
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti44D → N AA sequence (PubMed:843082).Curated1
Sequence conflicti44D → N AA sequence (PubMed:7142173).Curated1
Sequence conflicti52N → D AA sequence (PubMed:843082).Curated1
Sequence conflicti64N → G in CAA68697 (PubMed:2961612).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_03201112L → F in PCTT. 1 PublicationCorresponds to variant dbSNP:rs35877720EnsemblClinVar.1
Natural variantiVAR_01168814L → P in PCTT. 1 PublicationCorresponds to variant dbSNP:rs104893939EnsemblClinVar.1
Natural variantiVAR_01168934N → S in PCTT and TCP; associated with disease susceptibility; risk factor also for acute pancreatitis; may confer susceptibility to fibrocalculous pancreatic diabetes. 4 PublicationsCorresponds to variant dbSNP:rs17107315EnsemblClinVar.1
Natural variantiVAR_01169055P → S4 PublicationsCorresponds to variant dbSNP:rs111966833EnsemblClinVar.1
Natural variantiVAR_03201267R → H. Corresponds to variant dbSNP:rs35523678EnsemblClinVar.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M20530
, M22971, M20528, M20529 Genomic DNA Translation: AAA36522.1
Y00705 mRNA Translation: CAA68697.1
M11949 mRNA Translation: AAA36521.1
AF286028 Genomic DNA Translation: AAG00531.1
BC025790 mRNA Translation: AAH25790.1
CCDSiCCDS4286.1
PIRiA27484 TIHUA
RefSeqiNP_003113.2, NM_003122.4
UniGeneiHs.407856

Genome annotation databases

EnsembliENST00000296695; ENSP00000296695; ENSG00000164266
GeneIDi6690
KEGGihsa:6690
UCSCiuc003los.3 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Entry informationi

Entry nameiISK1_HUMAN
AccessioniPrimary (citable) accession number: P00995
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 21, 1986
Last sequence update: July 1, 1989
Last modified: May 23, 2018
This is version 186 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

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