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Protein

Serpin B6

Gene

SERPINB6

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

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 keyPosition(s)DescriptionActionsGraphical viewLength
Sitei341 – 342Reactive bond2

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
  • sensory perception of sound Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Protease inhibitor, Serine protease inhibitor

Enzyme and pathway databases

BioCyciZFISH:ENSG00000124570-MONOMER.
ReactomeiR-HSA-6798695. Neutrophil degranulation.
R-HSA-75205. Dissolution of Fibrin Clot.

Protein family/group databases

MEROPSiI04.011.

Names & Taxonomyi

Protein namesi
Recommended name:
Serpin B6
Alternative name(s):
Cytoplasmic antiproteinase
Short name:
CAP
Peptidase inhibitor 6
Short name:
PI-6
Placental thrombin inhibitor
Gene namesi
Name:SERPINB6
Synonyms:PI6, PTI
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 6

Organism-specific databases

HGNCiHGNC:8950. SERPINB6.

Subcellular locationi

GO - Cellular componenti

  • cytoplasm Source: UniProtKB
  • cytosol Source: ProtInc
  • extracellular exosome Source: UniProtKB
  • extracellular space Source: GO_Central
  • protein complex Source: BHF-UCL
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm

Pathology & Biotechi

Involvement in diseasei

Deafness, autosomal recessive, 91 (DFNB91)1 Publication
The disease is caused by mutations 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.
See also OMIM:613453

Keywords - Diseasei

Deafness, Non-syndromic deafness

Organism-specific databases

DisGeNETi5269.
MalaCardsiSERPINB6.
MIMi613453. phenotype.
OpenTargetsiENSG00000124570.
Orphaneti90636. Autosomal recessive non-syndromic sensorineural deafness type DFNB.
PharmGKBiPA35516.

Chemistry databases

DrugBankiDB00055. Drotrecogin alfa.

Polymorphism and mutation databases

BioMutaiSERPINB6.
DMDMi161784343.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000941061 – 376Serpin B6Add BLAST376

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei1N-acetylmethionineCombined sources1
Modified residuei151PhosphoserineCombined sources1
Modified residuei195N6-acetyllysineBy similarity1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiP35237.
MaxQBiP35237.
PaxDbiP35237.
PeptideAtlasiP35237.
PRIDEiP35237.

2D gel databases

OGPiP35237.

PTM databases

iPTMnetiP35237.
PhosphoSitePlusiP35237.
SwissPalmiP35237.

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

BgeeiENSG00000124570.
CleanExiHS_SERPINB6.
ExpressionAtlasiP35237. baseline and differential.
GenevisibleiP35237. HS.

Organism-specific databases

HPAiHPA009668.
HPA012736.

Interactioni

Subunit structurei

Forms a complex with the monomeric form of beta-tryptase.

GO - Molecular functioni

  • protease binding Source: UniProtKB

Protein-protein interaction databases

BioGridi111287. 13 interactors.
IntActiP35237. 1 interactor.
MINTiMINT-5005332.
STRINGi9606.ENSP00000338358.

Structurei

3D structure databases

ProteinModelPortaliP35237.
SMRiP35237.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

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

Phylogenomic databases

eggNOGiKOG2392. Eukaryota.
COG4826. LUCA.
GeneTreeiENSGT00760000118789.
HOGENOMiHOG000238519.
HOVERGENiHBG005957.
InParanoidiP35237.
KOiK13963.
PhylomeDBiP35237.
TreeFamiTF352619.

Family and domain databases

InterProiIPR023795. Serpin_CS.
IPR023796. Serpin_dom.
IPR000215. Serpin_fam.
[Graphical view]
PANTHERiPTHR11461. PTHR11461. 1 hit.
PfamiPF00079. Serpin. 1 hit.
[Graphical view]
SMARTiSM00093. SERPIN. 1 hit.
[Graphical view]
SUPFAMiSSF56574. SSF56574. 1 hit.
PROSITEiPS00284. SERPIN. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

P35237-1 [UniParc]FASTAAdd to basket

« Hide

        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
Length:376
Mass (Da):42,622
Last modified:December 4, 2007 - v3
Checksum:i3A06BC26A7510E1A
GO

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 keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti175E → G in CAA80373 (PubMed:8415716).Curated1
Sequence conflicti200M → V in BAG37155 (PubMed:14702039).Curated1
Sequence conflicti362S → R in CAA80373 (PubMed:8415716).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_03729590M → V.4 PublicationsCorresponds to variant rs2295769dbSNPEnsembl.1
Natural variantiVAR_037296153G → S.Corresponds to variant rs2295766dbSNPEnsembl.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. Translation: CAI23292.1.
CH471087 Genomic DNA. Translation: EAW55097.1.
BC001394 mRNA. Translation: AAH01394.1.
BC098564 mRNA. Translation: AAH98564.1.
AB209563 mRNA. Translation: BAD92800.1. Different initiation.
CCDSiCCDS4479.1.
PIRiA48681.
RefSeqiNP_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.
UniGeneiHs.519523.
Hs.731506.

Genome annotation databases

EnsembliENST00000335686; ENSP00000338358; ENSG00000124570.
ENST00000380520; ENSP00000369891; ENSG00000124570.
ENST00000380524; ENSP00000369896; ENSG00000124570.
ENST00000380529; ENSP00000369901; ENSG00000124570.
ENST00000380539; ENSP00000369912; ENSG00000124570.
ENST00000380546; ENSP00000369919; ENSG00000124570.
GeneIDi5269.
KEGGihsa:5269.
UCSCiuc003muk.3. human.

Keywords - Coding sequence diversityi

Polymorphism

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. Translation: CAI23292.1.
CH471087 Genomic DNA. Translation: EAW55097.1.
BC001394 mRNA. Translation: AAH01394.1.
BC098564 mRNA. Translation: AAH98564.1.
AB209563 mRNA. Translation: BAD92800.1. Different initiation.
CCDSiCCDS4479.1.
PIRiA48681.
RefSeqiNP_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.
UniGeneiHs.519523.
Hs.731506.

3D structure databases

ProteinModelPortaliP35237.
SMRiP35237.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi111287. 13 interactors.
IntActiP35237. 1 interactor.
MINTiMINT-5005332.
STRINGi9606.ENSP00000338358.

Chemistry databases

DrugBankiDB00055. Drotrecogin alfa.

Protein family/group databases

MEROPSiI04.011.

PTM databases

iPTMnetiP35237.
PhosphoSitePlusiP35237.
SwissPalmiP35237.

Polymorphism and mutation databases

BioMutaiSERPINB6.
DMDMi161784343.

2D gel databases

OGPiP35237.

Proteomic databases

EPDiP35237.
MaxQBiP35237.
PaxDbiP35237.
PeptideAtlasiP35237.
PRIDEiP35237.

Protocols and materials databases

DNASUi5269.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000335686; ENSP00000338358; ENSG00000124570.
ENST00000380520; ENSP00000369891; ENSG00000124570.
ENST00000380524; ENSP00000369896; ENSG00000124570.
ENST00000380529; ENSP00000369901; ENSG00000124570.
ENST00000380539; ENSP00000369912; ENSG00000124570.
ENST00000380546; ENSP00000369919; ENSG00000124570.
GeneIDi5269.
KEGGihsa:5269.
UCSCiuc003muk.3. human.

Organism-specific databases

CTDi5269.
DisGeNETi5269.
GeneCardsiSERPINB6.
H-InvDBHIX0005534.
HGNCiHGNC:8950. SERPINB6.
HPAiHPA009668.
HPA012736.
MalaCardsiSERPINB6.
MIMi173321. gene.
613453. phenotype.
neXtProtiNX_P35237.
OpenTargetsiENSG00000124570.
Orphaneti90636. Autosomal recessive non-syndromic sensorineural deafness type DFNB.
PharmGKBiPA35516.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2392. Eukaryota.
COG4826. LUCA.
GeneTreeiENSGT00760000118789.
HOGENOMiHOG000238519.
HOVERGENiHBG005957.
InParanoidiP35237.
KOiK13963.
PhylomeDBiP35237.
TreeFamiTF352619.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000124570-MONOMER.
ReactomeiR-HSA-6798695. Neutrophil degranulation.
R-HSA-75205. Dissolution of Fibrin Clot.

Miscellaneous databases

ChiTaRSiSERPINB6. human.
GeneWikiiSERPINB6.
GenomeRNAii5269.
PROiP35237.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000124570.
CleanExiHS_SERPINB6.
ExpressionAtlasiP35237. baseline and differential.
GenevisibleiP35237. HS.

Family and domain databases

InterProiIPR023795. Serpin_CS.
IPR023796. Serpin_dom.
IPR000215. Serpin_fam.
[Graphical view]
PANTHERiPTHR11461. PTHR11461. 1 hit.
PfamiPF00079. Serpin. 1 hit.
[Graphical view]
SMARTiSM00093. SERPIN. 1 hit.
[Graphical view]
SUPFAMiSSF56574. SSF56574. 1 hit.
PROSITEiPS00284. SERPIN. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiSPB6_HUMAN
AccessioniPrimary (citable) accession number: P35237
Secondary accession number(s): B2RBA8
, Q59F97, Q5TD06, Q7Z2Y7, Q96J44, Q9UDI7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 1, 1994
Last sequence update: December 4, 2007
Last modified: November 30, 2016
This is version 165 of the entry and version 3 of the sequence. [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

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 6
    Human chromosome 6: entries, gene names and cross-references to MIM
  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. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.