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Protein

Cystatin-A

Gene

CSTA

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

Functioni

This is an intracellular thiol proteinase inhibitor. Has an important role in desmosome-mediated cell-cell adhesion in the lower levels of the epidermis.1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sitei4Reactive site1

GO - Molecular functioni

  • cysteine-type endopeptidase inhibitor activity Source: UniProtKB
  • protease binding Source: BHF-UCL
  • protein binding, bridging Source: UniProtKB
  • structural molecule activity Source: UniProtKB

GO - Biological processi

  • cell-cell adhesion Source: UniProtKB
  • cornification Source: Reactome
  • keratinocyte differentiation Source: UniProtKB
  • negative regulation of peptidase activity Source: BHF-UCL
  • negative regulation of proteolysis Source: UniProtKB
  • peptide cross-linking Source: UniProtKB

Keywordsi

Molecular functionProtease inhibitor, Thiol protease inhibitor
Biological processCell adhesion

Enzyme and pathway databases

ReactomeiR-HSA-6809371. Formation of the cornified envelope.

Protein family/group databases

MEROPSiI25.001.

Names & Taxonomyi

Protein namesi
Recommended name:
Cystatin-A
Alternative name(s):
Cystatin-AS
Stefin-A
Cleaved into the following chain:
Gene namesi
Name:CSTA
Synonyms:STF1, STFA
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 3

Organism-specific databases

EuPathDBiHostDB:ENSG00000121552.3.
HGNCiHGNC:2481. CSTA.

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

Peeling skin syndrome 4 (PSS4)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA genodermatosis characterized by congenital exfoliative ichthyosis, sharing some features with ichthyosis bullosa of Siemens and annular epidermolytic ichthyosis. PSS4 presents shortly after birth as dry, scaly skin over most of the body with coarse peeling of non-erythematous skin on the palms and soles, which is exacerbated by excessive moisture and minor trauma. Electron microscopy analysis of skin biopsies, reveals mostly normal-appearing upper layers of the epidermis, but prominent intercellular edema of the basal and suprabasal cell layers with aggregates of tonofilaments in the basal keratinocytes.
See also OMIM:607936

Keywords - Diseasei

Ichthyosis

Organism-specific databases

DisGeNETi1475.
MalaCardsiCSTA.
MIMi607936. phenotype.
OpenTargetsiENSG00000121552.
Orphaneti289586. Exfoliative ichthyosis.
PharmGKBiPA26983.

Polymorphism and mutation databases

BioMutaiCSTA.
DMDMi118177.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00004232021 – 98Cystatin-AAdd BLAST98
Initiator methionineiRemoved; alternate1 Publication
ChainiPRO_00002071282 – 98Cystatin-A, N-terminally processedAdd BLAST97

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei1N-acetylmethionineBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiP01040.
PaxDbiP01040.
PeptideAtlasiP01040.
PRIDEiP01040.
TopDownProteomicsiP01040.

PTM databases

iPTMnetiP01040.
PhosphoSitePlusiP01040.

Expressioni

Tissue specificityi

Expressed in the skin throughout the epidermis.1 Publication

Gene expression databases

BgeeiENSG00000121552.
CleanExiHS_CSTA.
ExpressionAtlasiP01040. baseline and differential.
GenevisibleiP01040. HS.

Organism-specific databases

HPAiCAB000469.
CAB047315.
HPA001031.

Interactioni

GO - Molecular functioni

  • protease binding Source: BHF-UCL
  • protein binding, bridging Source: UniProtKB

Protein-protein interaction databases

BioGridi107857. 33 interactors.
IntActiP01040. 15 interactors.
MINTiMINT-1428155.
STRINGi9606.ENSP00000264474.

Structurei

Secondary structure

198
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi7 – 12Combined sources6
Helixi14 – 31Combined sources18
Beta strandi34 – 36Combined sources3
Beta strandi39 – 58Combined sources20
Helixi60 – 62Combined sources3
Beta strandi64 – 71Combined sources8
Helixi74 – 76Combined sources3
Helixi77 – 79Combined sources3
Beta strandi80 – 89Combined sources10
Beta strandi91 – 93Combined sources3

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1CYUNMR-A1-98[»]
1CYVNMR-A1-98[»]
1DVCNMR-A1-98[»]
1DVDNMR-A1-98[»]
1GD3NMR-A1-98[»]
1GD4NMR-A1-98[»]
1N9JNMR-A/B1-98[»]
1NB3X-ray2.80I/J/K/L1-98[»]
1NB5X-ray2.40I/J/K/L1-98[»]
3K9MX-ray2.61C/D1-98[»]
3KFQX-ray1.99C/D1-98[»]
3KSEX-ray1.71D/E/F1-98[»]
ProteinModelPortaliP01040.
SMRiP01040.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP01040.

Family & Domainsi

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi46 – 50Secondary area of contact5

Sequence similaritiesi

Belongs to the cystatin family.Curated

Phylogenomic databases

eggNOGiENOG410J1PC. Eukaryota.
ENOG41121QW. LUCA.
GeneTreeiENSGT00390000015607.
HOGENOMiHOG000294175.
HOVERGENiHBG002292.
InParanoidiP01040.
KOiK13907.
OMAiYQTDKSK.
OrthoDBiEOG091G12NK.
PhylomeDBiP01040.
TreeFamiTF333174.

Family and domain databases

CDDicd00042. CY. 1 hit.
InterProiView protein in InterPro
IPR000010. Cystatin_dom.
IPR018073. Prot_inh_cystat_CS.
IPR001713. Prot_inh_stefin.
PANTHERiPTHR11414. PTHR11414. 1 hit.
PfamiView protein in Pfam
PF00031. Cystatin. 1 hit.
PRINTSiPR00295. STEFINA.
SMARTiView protein in SMART
SM00043. CY. 1 hit.
PROSITEiView protein in PROSITE
PS00287. CYSTATIN. 1 hit.

Sequencei

Sequence statusi: Complete.

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

P01040-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MIPGGLSEAK PATPEIQEIV DKVKPQLEEK TNETYGKLEA VQYKTQVVAG
60 70 80 90
TNYYIKVRAG DNKYMHLKVF KSLPGQNEDL VLTGYQVDKN KDDELTGF
Length:98
Mass (Da):11,006
Last modified:July 21, 1986 - v1
Checksum:i2F5F0D61C91305EE
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_04885196T → M. Corresponds to variant dbSNP:rs34173813Ensembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X05978 mRNA. Translation: CAA29398.1.
D88422 Genomic DNA. Translation: BAA13609.1.
AB007774 Genomic DNA. Translation: BAA87858.1.
AK291308 mRNA. Translation: BAF83997.1.
CR456914 mRNA. Translation: CAG33195.1.
CH471052 Genomic DNA. Translation: EAW79489.1.
BC010379 mRNA. Translation: AAH10379.1.
CCDSiCCDS3011.1.
PIRiA29139. UDHUS.
RefSeqiNP_005204.1. NM_005213.3.
UniGeneiHs.518198.

Genome annotation databases

EnsembliENST00000264474; ENSP00000264474; ENSG00000121552.
GeneIDi1475.
KEGGihsa:1475.
UCSCiuc003eex.4. human.

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Entry informationi

Entry nameiCYTA_HUMAN
AccessioniPrimary (citable) accession number: P01040
Secondary accession number(s): Q6IB90
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 21, 1986
Last sequence update: July 21, 1986
Last modified: November 22, 2017
This is version 187 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. Human chromosome 3
    Human chromosome 3: 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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families