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Protein

Leukocyte elastase inhibitor

Gene

SERPINB1

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

Functioni

Regulates the activity of the neutrophil proteases elastase, cathepsin G, proteinase-3, chymase, chymotrypsin, and kallikrein-3. Also functions as a potent intracellular inhibitor of granzyme H.1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sitei343 – 344Reactive bond 12
Sitei344 – 345Reactive bond 22

GO - Molecular functioni

  • serine-type endopeptidase inhibitor activity Source: GO_Central

GO - Biological processi

Keywordsi

Molecular functionProtease inhibitor, Serine protease inhibitor

Enzyme and pathway databases

ReactomeiR-HSA-6798695 Neutrophil degranulation

Protein family/group databases

MEROPSiI04.006

Names & Taxonomyi

Protein namesi
Recommended name:
Leukocyte elastase inhibitor
Short name:
LEI
Alternative name(s):
Monocyte/neutrophil elastase inhibitor
Short name:
EI
Short name:
M/NEI
Peptidase inhibitor 2
Short name:
PI-2
Serpin B1
Gene namesi
Name:SERPINB1
Synonyms:ELANH2, MNEI, PI2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 6

Organism-specific databases

EuPathDBiHostDB:ENSG00000021355.12
HGNCiHGNC:3311 SERPINB1
MIMi130135 gene
neXtProtiNX_P30740

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

Cytoplasm

Pathology & Biotechi

Organism-specific databases

DisGeNETi1992
OpenTargetsiENSG00000021355
PharmGKBiPA35046

Polymorphism and mutation databases

DMDMi266344

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000941011 – 379Leukocyte elastase inhibitorAdd BLAST379

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei1N-acetylmethionineCombined sources1 Publication1
Modified residuei137N6-acetyllysineCombined sources1
Modified residuei177N6-acetyllysineCombined sources1
Modified residuei300PhosphoserineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiP30740
PaxDbiP30740
PeptideAtlasiP30740
PRIDEiP30740
ProteomicsDBi54734

2D gel databases

OGPiP30740
REPRODUCTION-2DPAGEiIPI00027444

PTM databases

iPTMnetiP30740
PhosphoSitePlusiP30740

Miscellaneous databases

PMAP-CutDBiP30740

Expressioni

Gene expression databases

BgeeiENSG00000021355 Expressed in 220 organ(s), highest expression level in esophagus squamous epithelium
CleanExiHS_SERPINB1
ExpressionAtlasiP30740 baseline and differential
GenevisibleiP30740 HS

Organism-specific databases

HPAiHPA018871
HPA018882
HPA052642

Interactioni

Subunit structurei

Monomer.1 Publication

Protein-protein interaction databases

BioGridi108307, 23 interactors
IntActiP30740, 3 interactors
STRINGi9606.ENSP00000370115

Structurei

Secondary structure

1379
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliP30740
SMRiP30740
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domaini

Reactive bond 1 is specific for reaction with chymotrypsin-like protease such as cathepsin G, chymotrypsin, chymase or granzyme H, while reactive bond 2 is specific for reaction with elastase-like protease such as neutrophil elastase, proteinase-3, pancreatic elastase or PSA.

Sequence similaritiesi

Belongs to the serpin family. Ov-serpin subfamily.Curated

Phylogenomic databases

eggNOGiKOG2392 Eukaryota
COG4826 LUCA
GeneTreeiENSGT00760000118789
HOGENOMiHOG000238519
HOVERGENiHBG005957
InParanoidiP30740
KOiK13963
OMAiGMVAFCM
OrthoDBiEOG091G0ION
PhylomeDBiP30740
TreeFamiTF352619

Family and domain databases

InterProiView protein in InterPro
IPR015557 Serpin_B1
IPR023795 Serpin_CS
IPR023796 Serpin_dom
IPR000215 Serpin_fam
IPR036186 Serpin_sf
PANTHERiPTHR11461 PTHR11461, 1 hit
PTHR11461:SF180 PTHR11461:SF180, 1 hit
PfamiView protein in Pfam
PF00079 Serpin, 1 hit
SMARTiView protein in SMART
SM00093 SERPIN, 1 hit
SUPFAMiSSF56574 SSF56574, 1 hit
PROSITEiView protein in PROSITE
PS00284 SERPIN, 1 hit

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket
Isoform 1 (identifier: P30740-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MEQLSSANTR FALDLFLALS ENNPAGNIFI SPFSISSAMA MVFLGTRGNT
60 70 80 90 100
AAQLSKTFHF NTVEEVHSRF QSLNADINKR GASYILKLAN RLYGEKTYNF
110 120 130 140 150
LPEFLVSTQK TYGADLASVD FQHASEDARK TINQWVKGQT EGKIPELLAS
160 170 180 190 200
GMVDNMTKLV LVNAIYFKGN WKDKFMKEAT TNAPFRLNKK DRKTVKMMYQ
210 220 230 240 250
KKKFAYGYIE DLKCRVLELP YQGEELSMVI LLPDDIEDES TGLKKIEEQL
260 270 280 290 300
TLEKLHEWTK PENLDFIEVN VSLPRFKLEE SYTLNSDLAR LGVQDLFNSS
310 320 330 340 350
KADLSGMSGA RDIFISKIVH KSFVEVNEEG TEAAAATAGI ATFCMLMPEE
360 370
NFTADHPFLF FIRHNSSGSI LFLGRFSSP
Length:379
Mass (Da):42,742
Last modified:April 1, 1993 - v1
Checksum:iBAAE08DFCBCD8CD3
GO
Isoform 2 (identifier: P30740-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-151: Missing.

Note: No experimental confirmation available.
Show »
Length:228
Mass (Da):26,073
Checksum:iEDA3D9952C365A47
GO

Sequence cautioni

The sequence BAD97090 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti137K → R in AAP35574 (Ref. 4) Curated1
Sequence conflicti149A → V in BAF84016 (PubMed:14702039).Curated1
Sequence conflicti264L → F in BAF84016 (PubMed:14702039).Curated1
Sequence conflicti272Missing AA sequence (PubMed:7578269).Curated1
Sequence conflicti329E → K in BAF84016 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_05194582A → V. Corresponds to variant dbSNP:rs34825616Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0565111 – 151Missing in isoform 2. 1 PublicationAdd BLAST151

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M93056 mRNA No translation available.
AF053630 Genomic DNA Translation: AAC31394.1
AK291327 mRNA Translation: BAF84016.1
AK298044 mRNA Translation: BAG60342.1
BT006928 mRNA Translation: AAP35574.1
AK223370 mRNA Translation: BAD97090.1 Different initiation.
AL139092 Genomic DNA No translation available.
BC009015 mRNA Translation: AAH09015.1
CCDSiCCDS4477.1 [P30740-1]
PIRiS27383
RefSeqiNP_109591.1, NM_030666.3 [P30740-1]
XP_011512635.1, XM_011514333.1 [P30740-1]
XP_011512636.1, XM_011514334.2 [P30740-1]
UniGeneiHs.381167

Genome annotation databases

EnsembliENST00000380739; ENSP00000370115; ENSG00000021355 [P30740-1]
GeneIDi1992
KEGGihsa:1992

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M93056 mRNA No translation available.
AF053630 Genomic DNA Translation: AAC31394.1
AK291327 mRNA Translation: BAF84016.1
AK298044 mRNA Translation: BAG60342.1
BT006928 mRNA Translation: AAP35574.1
AK223370 mRNA Translation: BAD97090.1 Different initiation.
AL139092 Genomic DNA No translation available.
BC009015 mRNA Translation: AAH09015.1
CCDSiCCDS4477.1 [P30740-1]
PIRiS27383
RefSeqiNP_109591.1, NM_030666.3 [P30740-1]
XP_011512635.1, XM_011514333.1 [P30740-1]
XP_011512636.1, XM_011514334.2 [P30740-1]
UniGeneiHs.381167

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
4GA7X-ray2.90A/B1-379[»]
ProteinModelPortaliP30740
SMRiP30740
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi108307, 23 interactors
IntActiP30740, 3 interactors
STRINGi9606.ENSP00000370115

Protein family/group databases

MEROPSiI04.006

PTM databases

iPTMnetiP30740
PhosphoSitePlusiP30740

Polymorphism and mutation databases

DMDMi266344

2D gel databases

OGPiP30740
REPRODUCTION-2DPAGEiIPI00027444

Proteomic databases

EPDiP30740
PaxDbiP30740
PeptideAtlasiP30740
PRIDEiP30740
ProteomicsDBi54734

Protocols and materials databases

DNASUi1992
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000380739; ENSP00000370115; ENSG00000021355 [P30740-1]
GeneIDi1992
KEGGihsa:1992

Organism-specific databases

CTDi1992
DisGeNETi1992
EuPathDBiHostDB:ENSG00000021355.12
GeneCardsiSERPINB1
HGNCiHGNC:3311 SERPINB1
HPAiHPA018871
HPA018882
HPA052642
MIMi130135 gene
neXtProtiNX_P30740
OpenTargetsiENSG00000021355
PharmGKBiPA35046
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2392 Eukaryota
COG4826 LUCA
GeneTreeiENSGT00760000118789
HOGENOMiHOG000238519
HOVERGENiHBG005957
InParanoidiP30740
KOiK13963
OMAiGMVAFCM
OrthoDBiEOG091G0ION
PhylomeDBiP30740
TreeFamiTF352619

Enzyme and pathway databases

ReactomeiR-HSA-6798695 Neutrophil degranulation

Miscellaneous databases

ChiTaRSiSERPINB1 human
GeneWikiiSERPINB1
GenomeRNAii1992
PMAP-CutDBiP30740
PROiPR:P30740
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000021355 Expressed in 220 organ(s), highest expression level in esophagus squamous epithelium
CleanExiHS_SERPINB1
ExpressionAtlasiP30740 baseline and differential
GenevisibleiP30740 HS

Family and domain databases

InterProiView protein in InterPro
IPR015557 Serpin_B1
IPR023795 Serpin_CS
IPR023796 Serpin_dom
IPR000215 Serpin_fam
IPR036186 Serpin_sf
PANTHERiPTHR11461 PTHR11461, 1 hit
PTHR11461:SF180 PTHR11461:SF180, 1 hit
PfamiView protein in Pfam
PF00079 Serpin, 1 hit
SMARTiView protein in SMART
SM00093 SERPIN, 1 hit
SUPFAMiSSF56574 SSF56574, 1 hit
PROSITEiView protein in PROSITE
PS00284 SERPIN, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiILEU_HUMAN
AccessioniPrimary (citable) accession number: P30740
Secondary accession number(s): A8K5L2
, B4DNT0, Q53FB9, Q5W0E1, Q9UDF8
Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 1, 1993
Last sequence update: April 1, 1993
Last modified: November 7, 2018
This is version 179 of the entry and version 1 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

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. Human chromosome 6
    Human chromosome 6: entries, gene names and cross-references to MIM
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Main funding by: National Institutes of Health

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