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Protein

Serpin B6

Gene

SERPINB6

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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
  • neutrophil degranulation Source: Reactome
  • sensory perception of sound Source: UniProtKB

Keywordsi

Molecular functionProtease inhibitor, Serine protease inhibitor

Enzyme and pathway databases

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

EuPathDBiHostDB:ENSG00000124570.17
HGNCiHGNC:8950 SERPINB6
MIMi173321 gene
neXtProtiNX_P35237

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

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, 14 interactors
IntActiP35237, 1 interactor
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

InterProiView protein in InterPro
IPR023795 Serpin_CS
IPR023796 Serpin_dom
IPR000215 Serpin_fam
IPR036186 Serpin_sf
PANTHERiPTHR11461 PTHR11461, 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

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 → V4 PublicationsCorresponds to variant dbSNP:rs2295769EnsemblClinVar.1
Natural variantiVAR_037296153G → 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.
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

Similar proteinsi

Entry informationi

Entry nameiSPB6_HUMAN
AccessioniPrimary (citable) accession number: P35237
Secondary accession number(s): B2RBA8
, Q59F97, Q5TD06, Q7Z2Y7, Q96J44, Q9UDI7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 1, 1994
Last sequence update: December 4, 2007
Last modified: May 23, 2018
This is version 174 of the entry and version 3 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

Complete proteome, Direct protein sequencing, Reference proteome

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