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Protein

Granzyme H

Gene

GZMH

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

Functioni

Cytotoxic chymotrypsin-like serine protease with preference for bulky and aromatic residues at the P1 position and acidic residues at the P3' and P4' sites. Probably necessary for target cell lysis in cell-mediated immune responses. Participates in the antiviral response via direct cleavage of several proteins essential for viral replication.2 Publications

Activity regulationi

Inhibited by SERPINB1.1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei64Charge relay systemBy similarity1
Active sitei108Charge relay systemBy similarity1
Active sitei202Charge relay systemBy similarity1

GO - Molecular functioni

  • serine-type endopeptidase activity Source: UniProtKB

GO - Biological processi

  • apoptotic process Source: UniProtKB
  • cytolysis Source: UniProtKB-KW
  • granzyme-mediated apoptotic signaling pathway Source: GO_Central

Keywordsi

Molecular functionHydrolase, Protease, Serine protease
Biological processCytolysis

Enzyme and pathway databases

ReactomeiR-HSA-2022377 Metabolism of Angiotensinogen to Angiotensins
R-HSA-381426 Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs)

Protein family/group databases

MEROPSiS01.147

Names & Taxonomyi

Protein namesi
Recommended name:
Granzyme H (EC:3.4.21.-)
Alternative name(s):
CCP-X
Cathepsin G-like 2
Short name:
CTSGL2
Cytotoxic T-lymphocyte proteinase
Cytotoxic serine protease C
Short name:
CSP-C
Gene namesi
Name:GZMH
Synonyms:CGL2, CTSGL2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 14

Organism-specific databases

EuPathDBiHostDB:ENSG00000100450.12
HGNCiHGNC:4710 GZMH
MIMi116831 gene
neXtProtiNX_P20718

Subcellular locationi

Pathology & Biotechi

Organism-specific databases

DisGeNETi2999
OpenTargetsiENSG00000100450
PharmGKBiPA29088

Polymorphism and mutation databases

DMDMi121590

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 18Add BLAST18
PropeptideiPRO_000002741319 – 20Activation peptide2
ChainiPRO_000002741421 – 246Granzyme HAdd BLAST226

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Disulfide bondi49 ↔ 65
Glycosylationi71N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi104N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi142 ↔ 208
Disulfide bondi172 ↔ 187
Glycosylationi179N-linked (GlcNAc...) asparagineSequence analysis1

Keywords - PTMi

Disulfide bond, Glycoprotein, Zymogen

Proteomic databases

PaxDbiP20718
PeptideAtlasiP20718
PRIDEiP20718
ProteomicsDBi53779

PTM databases

GlyConnecti1291
PhosphoSitePlusiP20718

Expressioni

Tissue specificityi

Constitutively expressed in NK cells.1 Publication

Gene expression databases

BgeeiENSG00000100450 Expressed in 124 organ(s), highest expression level in mononuclear cell
CleanExiHS_GZMH
ExpressionAtlasiP20718 baseline and differential
GenevisibleiP20718 HS

Organism-specific databases

HPAiHPA029200

Interactioni

Protein-protein interaction databases

BioGridi109254, 1 interactor
IntActiP20718, 1 interactor
MINTiP20718
STRINGi9606.ENSP00000216338

Structurei

Secondary structure

1246
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliP20718
SMRiP20718
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini21 – 244Peptidase S1PROSITE-ProRule annotationAdd BLAST224

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni46 – 48Mediates the preference for acidic residues at the P3' and P4' sites3

Sequence similaritiesi

Belongs to the peptidase S1 family. Granzyme subfamily.PROSITE-ProRule annotation

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiKOG3627 Eukaryota
COG5640 LUCA
GeneTreeiENSGT00760000118895
HOGENOMiHOG000251820
HOVERGENiHBG013304
InParanoidiP20718
KOiK09616
OMAiPWIKRIM
OrthoDBiEOG091G0G5F
PhylomeDBiP20718
TreeFamiTF333630

Family and domain databases

CDDicd00190 Tryp_SPc, 1 hit
InterProiView protein in InterPro
IPR009003 Peptidase_S1_PA
IPR001314 Peptidase_S1A
IPR001254 Trypsin_dom
IPR018114 TRYPSIN_HIS
IPR033116 TRYPSIN_SER
PfamiView protein in Pfam
PF00089 Trypsin, 1 hit
PRINTSiPR00722 CHYMOTRYPSIN
SMARTiView protein in SMART
SM00020 Tryp_SPc, 1 hit
SUPFAMiSSF50494 SSF50494, 1 hit
PROSITEiView protein in PROSITE
PS50240 TRYPSIN_DOM, 1 hit
PS00134 TRYPSIN_HIS, 1 hit
PS00135 TRYPSIN_SER, 1 hit

Sequences (3+)i

Sequence statusi: Complete.

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

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 3 described isoforms and 1 potential isoform that is computationally mapped.Show allAlign All

Isoform 1 (identifier: P20718-1) [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
MQPFLLLLAF LLTPGAGTEE IIGGHEAKPH SRPYMAFVQF LQEKSRKRCG
60 70 80 90 100
GILVRKDFVL TAAHCQGSSI NVTLGAHNIK EQERTQQFIP VKRPIPHPAY
110 120 130 140 150
NPKNFSNDIM LLQLERKAKW TTAVRPLRLP SSKAQVKPGQ LCSVAGWGYV
160 170 180 190 200
SMSTLATTLQ EVLLTVQKDC QCERLFHGNY SRATEICVGD PKKTQTGFKG
210 220 230 240
DSGGPLVCKD VAQGILSYGN KKGTPPGVYI KVSHFLPWIK RTMKRL
Length:246
Mass (Da):27,315
Last modified:February 1, 1991 - v1
Checksum:iCA6A87F3DA5F1E71
GO
Isoform 2 (identifier: P20718-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     114-199: Missing.

Show »
Length:160
Mass (Da):17,790
Checksum:i5EC04B6A4EAE8E19
GO
Isoform 3 (identifier: P20718-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     69-199: Missing.

Show »
Length:115
Mass (Da):12,609
Checksum:i06D2C85F733515FC
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A0C4DGJ9A0A0C4DGJ9_HUMAN
Granzyme H
GZMH
115Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti68S → R in AAP70248 (Ref. 4) Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_01455684R → Q. Corresponds to variant dbSNP:rs20545Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_04757369 – 199Missing in isoform 3. 1 PublicationAdd BLAST131
Alternative sequenceiVSP_047073114 – 199Missing in isoform 2. 1 PublicationAdd BLAST86

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J02907 Genomic DNA Translation: AAA76859.1
M57888 Genomic DNA Translation: AAA03514.1
M36118 mRNA Translation: AAA03248.1
M72150 Genomic DNA Translation: AAA74885.1
AY232657 mRNA Translation: AAP70247.1
AY232658 mRNA Translation: AAP70248.1
AL136018 Genomic DNA No translation available.
CH471078 Genomic DNA Translation: EAW66004.1
BC027974 mRNA Translation: AAH27974.1
CCDSiCCDS59243.1 [P20718-2]
CCDS9632.1 [P20718-1]
PIRiA32692
RefSeqiNP_001257710.1, NM_001270781.1 [P20718-2]
NP_219491.1, NM_033423.4 [P20718-1]
UniGeneiHs.348264

Genome annotation databases

EnsembliENST00000216338; ENSP00000216338; ENSG00000100450 [P20718-1]
ENST00000382548; ENSP00000371988; ENSG00000100450 [P20718-2]
GeneIDi2999
KEGGihsa:2999
UCSCiuc001wpr.3 human [P20718-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J02907 Genomic DNA Translation: AAA76859.1
M57888 Genomic DNA Translation: AAA03514.1
M36118 mRNA Translation: AAA03248.1
M72150 Genomic DNA Translation: AAA74885.1
AY232657 mRNA Translation: AAP70247.1
AY232658 mRNA Translation: AAP70248.1
AL136018 Genomic DNA No translation available.
CH471078 Genomic DNA Translation: EAW66004.1
BC027974 mRNA Translation: AAH27974.1
CCDSiCCDS59243.1 [P20718-2]
CCDS9632.1 [P20718-1]
PIRiA32692
RefSeqiNP_001257710.1, NM_001270781.1 [P20718-2]
NP_219491.1, NM_033423.4 [P20718-1]
UniGeneiHs.348264

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1LXVmodel-A21-246[»]
3TJUX-ray2.70A21-246[»]
3TJVX-ray2.40A21-246[»]
3TK9X-ray2.20A21-246[»]
4GAWX-ray3.00A/B/C/D/E/F/G/H/I/J/K/L21-246[»]
ProteinModelPortaliP20718
SMRiP20718
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi109254, 1 interactor
IntActiP20718, 1 interactor
MINTiP20718
STRINGi9606.ENSP00000216338

Protein family/group databases

MEROPSiS01.147

PTM databases

GlyConnecti1291
PhosphoSitePlusiP20718

Polymorphism and mutation databases

DMDMi121590

Proteomic databases

PaxDbiP20718
PeptideAtlasiP20718
PRIDEiP20718
ProteomicsDBi53779

Protocols and materials databases

DNASUi2999
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000216338; ENSP00000216338; ENSG00000100450 [P20718-1]
ENST00000382548; ENSP00000371988; ENSG00000100450 [P20718-2]
GeneIDi2999
KEGGihsa:2999
UCSCiuc001wpr.3 human [P20718-1]

Organism-specific databases

CTDi2999
DisGeNETi2999
EuPathDBiHostDB:ENSG00000100450.12
GeneCardsiGZMH
HGNCiHGNC:4710 GZMH
HPAiHPA029200
MIMi116831 gene
neXtProtiNX_P20718
OpenTargetsiENSG00000100450
PharmGKBiPA29088
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3627 Eukaryota
COG5640 LUCA
GeneTreeiENSGT00760000118895
HOGENOMiHOG000251820
HOVERGENiHBG013304
InParanoidiP20718
KOiK09616
OMAiPWIKRIM
OrthoDBiEOG091G0G5F
PhylomeDBiP20718
TreeFamiTF333630

Enzyme and pathway databases

ReactomeiR-HSA-2022377 Metabolism of Angiotensinogen to Angiotensins
R-HSA-381426 Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs)

Miscellaneous databases

GeneWikiiGZMH
GenomeRNAii2999
PROiPR:P20718
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000100450 Expressed in 124 organ(s), highest expression level in mononuclear cell
CleanExiHS_GZMH
ExpressionAtlasiP20718 baseline and differential
GenevisibleiP20718 HS

Family and domain databases

CDDicd00190 Tryp_SPc, 1 hit
InterProiView protein in InterPro
IPR009003 Peptidase_S1_PA
IPR001314 Peptidase_S1A
IPR001254 Trypsin_dom
IPR018114 TRYPSIN_HIS
IPR033116 TRYPSIN_SER
PfamiView protein in Pfam
PF00089 Trypsin, 1 hit
PRINTSiPR00722 CHYMOTRYPSIN
SMARTiView protein in SMART
SM00020 Tryp_SPc, 1 hit
SUPFAMiSSF50494 SSF50494, 1 hit
PROSITEiView protein in PROSITE
PS50240 TRYPSIN_DOM, 1 hit
PS00134 TRYPSIN_HIS, 1 hit
PS00135 TRYPSIN_SER, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiGRAH_HUMAN
AccessioniPrimary (citable) accession number: P20718
Secondary accession number(s): G3V2C5, Q6XGZ0, Q6XGZ1
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 1, 1991
Last sequence update: February 1, 1991
Last modified: November 7, 2018
This is version 165 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, 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. Peptidase families
    Classification of peptidase families and list of entries
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. Human chromosome 14
    Human chromosome 14: entries, gene names and cross-references to MIM
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