UniProtKB - P35237 (SPB6_HUMAN)
Protein
Serpin B6
Gene
SERPINB6
Organism
Homo sapiens (Human)
Status
Functioni
May be involved in the regulation of serine proteinases present in the brain or extravasated from the blood (By similarity). Inhibitor of cathepsin G, kallikrein-8 and thrombin. May play an important role in the inner ear in the protection against leakage of lysosomal content during stress and loss of this protection results in cell death and sensorineural hearing loss.By similarity5 Publications
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sitei | 341 – 342 | Reactive bond | 2 |
GO - Molecular functioni
- protease binding Source: UniProtKB
- serine-type endopeptidase inhibitor activity Source: BHF-UCL
GO - Biological processi
- cellular response to osmotic stress Source: UniProtKB
- negative regulation of endopeptidase activity Source: BHF-UCL
- neutrophil degranulation Source: Reactome
- sensory perception of sound Source: UniProtKB
Keywordsi
Molecular function | Protease inhibitor, Serine protease inhibitor |
Enzyme and pathway databases
PathwayCommonsi | P35237 |
Reactomei | R-HSA-6798695, Neutrophil degranulation R-HSA-75205, Dissolution of Fibrin Clot |
Protein family/group databases
MEROPSi | I04.011 |
Names & Taxonomyi
Protein namesi | Recommended name: Serpin B6Alternative name(s): Cytoplasmic antiproteinase Short name: CAP Peptidase inhibitor 6 Short name: PI-6 Placental thrombin inhibitor |
Gene namesi | Name:SERPINB6 Synonyms:PI6, PTI |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:8950, SERPINB6 |
MIMi | 173321, gene |
neXtProti | NX_P35237 |
VEuPathDBi | HostDB:ENSG00000124570.17 |
Subcellular locationi
Other locations
- Cytoplasm 4 Publications
Cytosol
- cytosol Source: ProtInc
Extracellular region or secreted
- collagen-containing extracellular matrix Source: BHF-UCL
- extracellular exosome Source: UniProtKB
- extracellular region Source: BHF-UCL
- extracellular space Source: GO_Central
Plasma Membrane
- plasma membrane Source: Reactome
Other locations
- cytoplasm Source: UniProtKB
- ficolin-1-rich granule membrane Source: Reactome
- secretory granule membrane Source: Reactome
- serine protease inhibitor complex Source: BHF-UCL
- tertiary granule membrane Source: Reactome
Keywords - Cellular componenti
CytoplasmPathology & Biotechi
Involvement in diseasei
Deafness, autosomal recessive, 91 (DFNB91)1 Publication
The disease is caused by variants affecting the gene represented in this entry.
Disease descriptionA form of non-syndromic deafness characterized by progressive and age-dependent sensorineural hearing loss. Vestibular function is normal.
Related information in OMIMKeywords - Diseasei
Deafness, Non-syndromic deafnessOrganism-specific databases
DisGeNETi | 5269 |
MalaCardsi | SERPINB6 |
MIMi | 613453, phenotype |
OpenTargetsi | ENSG00000124570 |
Orphaneti | 90636, Autosomal recessive non-syndromic sensorineural deafness type DFNB |
PharmGKBi | PA35516 |
Miscellaneous databases
Pharosi | P35237, Tbio |
Chemistry databases
DrugBanki | DB00055, Drotrecogin alfa |
Genetic variation databases
BioMutai | SERPINB6 |
DMDMi | 161784343 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
ChainiPRO_0000094106 | 1 – 376 | Serpin B6Add BLAST | 376 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 1 | N-acetylmethionineCombined sources | 1 | |
Modified residuei | 151 | PhosphoserineCombined sources | 1 | |
Modified residuei | 195 | N6-acetyllysineBy similarity | 1 |
Keywords - PTMi
Acetylation, PhosphoproteinProteomic databases
EPDi | P35237 |
jPOSTi | P35237 |
MassIVEi | P35237 |
MaxQBi | P35237 |
PaxDbi | P35237 |
PeptideAtlasi | P35237 |
PRIDEi | P35237 |
ProteomicsDBi | 54998 |
2D gel databases
OGPi | P35237 |
PTM databases
iPTMneti | P35237 |
MetOSitei | P35237 |
PhosphoSitePlusi | P35237 |
SwissPalmi | P35237 |
Expressioni
Tissue specificityi
Expressed in keratinocytes (at protein level). Highest levels in skeletal muscle. Also found in placenta, cardiac muscle, lung, liver, kidney and pancreas. Expressed in the inner ear hair cells. Expressed abundantly by normal mast cells in different tissues and by mast cells in mastocytoma lesions.3 Publications
Gene expression databases
Bgeei | ENSG00000124570, Expressed in pancreas and 238 other tissues |
ExpressionAtlasi | P35237, baseline and differential |
Genevisiblei | P35237, HS |
Organism-specific databases
HPAi | ENSG00000124570, Low tissue specificity |
Interactioni
Subunit structurei
Forms a complex with the monomeric form of beta-tryptase.
GO - Molecular functioni
- protease binding Source: UniProtKB
Protein-protein interaction databases
BioGRIDi | 111287, 22 interactors |
IntActi | P35237, 5 interactors |
STRINGi | 9606.ENSP00000484343 |
Miscellaneous databases
RNActi | P35237, protein |
Family & Domainsi
Sequence similaritiesi
Phylogenomic databases
eggNOGi | KOG2392, Eukaryota |
GeneTreei | ENSGT00940000154519 |
HOGENOMi | CLU_023330_0_2_1 |
InParanoidi | P35237 |
OrthoDBi | 1124079at2759 |
PhylomeDBi | P35237 |
TreeFami | TF352619 |
Family and domain databases
Gene3Di | 2.30.39.10, 1 hit 3.30.497.10, 1 hit |
InterProi | View protein in InterPro IPR023795, Serpin_CS IPR023796, Serpin_dom IPR000215, Serpin_fam IPR036186, Serpin_sf IPR042178, Serpin_sf_1 IPR042185, Serpin_sf_2 |
PANTHERi | PTHR11461, PTHR11461, 1 hit |
Pfami | View protein in Pfam PF00079, Serpin, 1 hit |
SMARTi | View protein in SMART SM00093, SERPIN, 1 hit |
SUPFAMi | SSF56574, SSF56574, 1 hit |
PROSITEi | View protein in PROSITE PS00284, SERPIN, 1 hit |
(1+)i Sequence
Sequence statusi: Complete.
This entry has 1 described isoform and 11 potential isoforms that are computationally mapped.Show allAlign All
P35237-1 [UniParc]FASTAAdd to basket
10 20 30 40 50
MDVLAEANGT FALNLLKTLG KDNSKNVFFS PMSMSCALAM VYMGAKGNTA
60 70 80 90 100
AQMAQILSFN KSGGGGDIHQ GFQSLLTEVN KTGTQYLLRM ANRLFGEKSC
110 120 130 140 150
DFLSSFRDSC QKFYQAEMEE LDFISAVEKS RKHINTWVAE KTEGKIAELL
160 170 180 190 200
SPGSVDPLTR LVLVNAVYFR GNWDEQFDKE NTEERLFKVS KNEEKPVQMM
210 220 230 240 250
FKQSTFKKTY IGEIFTQILV LPYVGKELNM IIMLPDETTD LRTVEKELTY
260 270 280 290 300
EKFVEWTRLD MMDEEEVEVS LPRFKLEESY DMESVLRNLG MTDAFELGKA
310 320 330 340 350
DFSGMSQTDL SLSKVVHKSF VEVNEEGTEA AAATAAIMMM RCARFVPRFC
360 370
ADHPFLFFIQ HSKTNGILFC GRFSSP
Computationally mapped potential isoform sequencesi
There are 11 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketA0A2R8Y5S1 | A0A2R8Y5S1_HUMAN | Serpin B6 | SERPINB6 | 60 | Annotation score: | ||
A0A2R8YDD0 | A0A2R8YDD0_HUMAN | Serpin B6 | SERPINB6 | 61 | Annotation score: | ||
A0A087X1N8 | A0A087X1N8_HUMAN | Serpin B6 | SERPINB6 hCG_22875 | 395 | Annotation score: | ||
A0A024QZX5 | A0A024QZX5_HUMAN | Serpin B6 | SERPINB6 hCG_22875 | 380 | Annotation score: | ||
A0A2R8YD12 | A0A2R8YD12_HUMAN | Serpin B6 | SERPINB6 | 332 | Annotation score: | ||
A0A2R8Y6A7 | A0A2R8Y6A7_HUMAN | Serpin B6 | SERPINB6 | 171 | Annotation score: | ||
A0A2R8Y517 | A0A2R8Y517_HUMAN | Serpin B6 | SERPINB6 | 77 | Annotation score: | ||
A0A2R8Y6N4 | A0A2R8Y6N4_HUMAN | Serpin B6 | SERPINB6 | 81 | Annotation score: | ||
A0A2R8Y7E5 | A0A2R8Y7E5_HUMAN | Serpin B6 | SERPINB6 | 17 | Annotation score: | ||
A0A2R8Y5Y8 | A0A2R8Y5Y8_HUMAN | Serpin B6 | SERPINB6 | 11 | Annotation score: | ||
There is more potential isoformShow all |
Sequence cautioni
The sequence BAD92800 differs from that shown. Reason: Erroneous initiation.Curated
The sequence CAD98106 differs from that shown. Reason: Erroneous initiation.Curated
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 175 | E → G in CAA80373 (PubMed:8415716).Curated | 1 | |
Sequence conflicti | 200 | M → V in BAG37155 (PubMed:14702039).Curated | 1 | |
Sequence conflicti | 362 | S → R in CAA80373 (PubMed:8415716).Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_037295 | 90 | M → V4 PublicationsCorresponds to variant dbSNP:rs2295769EnsemblClinVar. | 1 | |
Natural variantiVAR_037296 | 153 | G → S. Corresponds to variant dbSNP:rs2295766EnsemblClinVar. | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | Z22658 mRNA Translation: CAA80373.1 S69272 mRNA Translation: AAB30320.1 AK314578 mRNA Translation: BAG37155.1 BX538343 mRNA Translation: CAD98106.1 Different initiation. AL133351 Genomic DNA No translation available. CH471087 Genomic DNA Translation: EAW55097.1 BC001394 mRNA Translation: AAH01394.1 BC098564 mRNA Translation: AAH98564.1 AB209563 mRNA Translation: BAD92800.1 Different initiation. |
CCDSi | CCDS4479.1 |
PIRi | A48681 |
RefSeqi | NP_001182220.2, NM_001195291.2 NP_001258751.1, NM_001271822.1 NP_001258752.1, NM_001271823.1 NP_001258753.1, NM_001271824.1 NP_001258754.1, NM_001271825.1 NP_001284628.1, NM_001297699.1 NP_001284629.1, NM_001297700.1 NP_004559.4, NM_004568.5 XP_011512975.1, XM_011514673.1 XP_011512976.1, XM_011514674.2 |
Genome annotation databases
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | Z22658 mRNA Translation: CAA80373.1 S69272 mRNA Translation: AAB30320.1 AK314578 mRNA Translation: BAG37155.1 BX538343 mRNA Translation: CAD98106.1 Different initiation. AL133351 Genomic DNA No translation available. CH471087 Genomic DNA Translation: EAW55097.1 BC001394 mRNA Translation: AAH01394.1 BC098564 mRNA Translation: AAH98564.1 AB209563 mRNA Translation: BAD92800.1 Different initiation. |
CCDSi | CCDS4479.1 |
PIRi | A48681 |
RefSeqi | NP_001182220.2, NM_001195291.2 NP_001258751.1, NM_001271822.1 NP_001258752.1, NM_001271823.1 NP_001258753.1, NM_001271824.1 NP_001258754.1, NM_001271825.1 NP_001284628.1, NM_001297699.1 NP_001284629.1, NM_001297700.1 NP_004559.4, NM_004568.5 XP_011512975.1, XM_011514673.1 XP_011512976.1, XM_011514674.2 |
3D structure databases
SMRi | P35237 |
ModBasei | Search... |
Protein-protein interaction databases
BioGRIDi | 111287, 22 interactors |
IntActi | P35237, 5 interactors |
STRINGi | 9606.ENSP00000484343 |
Chemistry databases
DrugBanki | DB00055, Drotrecogin alfa |
Protein family/group databases
MEROPSi | I04.011 |
PTM databases
iPTMneti | P35237 |
MetOSitei | P35237 |
PhosphoSitePlusi | P35237 |
SwissPalmi | P35237 |
Genetic variation databases
BioMutai | SERPINB6 |
DMDMi | 161784343 |
2D gel databases
OGPi | P35237 |
Proteomic databases
EPDi | P35237 |
jPOSTi | P35237 |
MassIVEi | P35237 |
MaxQBi | P35237 |
PaxDbi | P35237 |
PeptideAtlasi | P35237 |
PRIDEi | P35237 |
ProteomicsDBi | 54998 |
Protocols and materials databases
Antibodypediai | 1702, 290 antibodies |
DNASUi | 5269 |
Genome annotation databases
Organism-specific databases
CTDi | 5269 |
DisGeNETi | 5269 |
GeneCardsi | SERPINB6 |
HGNCi | HGNC:8950, SERPINB6 |
HPAi | ENSG00000124570, Low tissue specificity |
MalaCardsi | SERPINB6 |
MIMi | 173321, gene 613453, phenotype |
neXtProti | NX_P35237 |
OpenTargetsi | ENSG00000124570 |
Orphaneti | 90636, Autosomal recessive non-syndromic sensorineural deafness type DFNB |
PharmGKBi | PA35516 |
VEuPathDBi | HostDB:ENSG00000124570.17 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG2392, Eukaryota |
GeneTreei | ENSGT00940000154519 |
HOGENOMi | CLU_023330_0_2_1 |
InParanoidi | P35237 |
OrthoDBi | 1124079at2759 |
PhylomeDBi | P35237 |
TreeFami | TF352619 |
Enzyme and pathway databases
PathwayCommonsi | P35237 |
Reactomei | R-HSA-6798695, Neutrophil degranulation R-HSA-75205, Dissolution of Fibrin Clot |
Miscellaneous databases
BioGRID-ORCSi | 5269, 13 hits in 874 CRISPR screens |
ChiTaRSi | SERPINB6, human |
GeneWikii | SERPINB6 |
GenomeRNAii | 5269 |
Pharosi | P35237, Tbio |
PROi | PR:P35237 |
RNActi | P35237, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000124570, Expressed in pancreas and 238 other tissues |
ExpressionAtlasi | P35237, baseline and differential |
Genevisiblei | P35237, HS |
Family and domain databases
Gene3Di | 2.30.39.10, 1 hit 3.30.497.10, 1 hit |
InterProi | View protein in InterPro IPR023795, Serpin_CS IPR023796, Serpin_dom IPR000215, Serpin_fam IPR036186, Serpin_sf IPR042178, Serpin_sf_1 IPR042185, Serpin_sf_2 |
PANTHERi | PTHR11461, PTHR11461, 1 hit |
Pfami | View protein in Pfam PF00079, Serpin, 1 hit |
SMARTi | View protein in SMART SM00093, SERPIN, 1 hit |
SUPFAMi | SSF56574, SSF56574, 1 hit |
PROSITEi | View protein in PROSITE PS00284, SERPIN, 1 hit |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | SPB6_HUMAN | |
Accessioni | P35237Primary (citable) accession number: P35237 Secondary accession number(s): B2RBA8 Q9UDI7 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | February 1, 1994 |
Last sequence update: | December 4, 2007 | |
Last modified: | February 10, 2021 | |
This is version 195 of the entry and version 3 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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
Direct protein sequencing, Reference proteomeDocuments
- Human chromosome 6
Human chromosome 6: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - SIMILARITY comments
Index of protein domains and families