InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: CTH

Summary

InnateDB Protein

IDBP-99661.7

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

CTH

Protein Name

cystathionase (cystathionine gamma-lyase)

Synonyms

Species

Homo sapiens

Ensembl Protein

ENSP00000311554

InnateDB Gene

IDBG-99657 (CTH)

Protein Structure

UniProt Annotation

Function

Catalyzes the last step in the trans-sulfuration pathway from methionine to cysteine. Has broad substrate specificity. Converts cystathionine to cysteine, ammonia and 2-oxobutanoate. Converts two cysteine molecules to lanthionine and hydrogen sulfide. Can also accept homocysteine as substrate. Specificity depends on the levels of the endogenous substrates. Generates the endogenous signaling molecule hydrogen sulfide (H2S), and so contributes to the regulation of blood pressure. Acts as a cysteine-protein sulfhydrase by mediating sulfhydration of target proteins: sulfhydration consists of converting -SH groups into -SSH on specific cysteine residues of target proteins such as GAPDH, PTPN1 and NF-kappa-B subunit RELA, thereby regulating their function. {ECO:0000269PubMed:19019829, ECO:0000269PubMed:19261609, ECO:0000269PubMed:22169477}.

Subcellular Localization

Cytoplasm.

Disease Associations

Cystathioninuria (CSTNU) [MIM:219500]: Autosomal recessive phenotype characterized by abnormal accumulation of plasma cystathionine, leading to increased urinary excretion. {ECO:0000269PubMed:12574942}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 10 experimentally validated interaction(s) in this database.

Experimentally validated
Total	10 [view]
Protein-Protein	10 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0004123	cystathionine gamma-lyase activity
GO:0005515	protein binding
GO:0005516	calmodulin binding
GO:0016846	carbon-sulfur lyase activity
GO:0030170	pyridoxal phosphate binding
GO:0044540	L-cystine L-cysteine-lyase (deaminating)
GO:0047982	homocysteine desulfhydrase activity
GO:0080146	L-cysteine desulfhydrase activity

Biological Process

GO:0000096	sulfur amino acid metabolic process
GO:0000098	sulfur amino acid catabolic process
GO:0006534	cysteine metabolic process
GO:0018272	protein-pyridoxal-5-phosphate linkage via peptidyl-N6-pyridoxal phosphate-L-lysine
GO:0019344	cysteine biosynthetic process
GO:0019346	transsulfuration
GO:0030968	endoplasmic reticulum unfolded protein response
GO:0034641	cellular nitrogen compound metabolic process
GO:0044281	small molecule metabolic process
GO:0044524	protein sulfhydration
GO:0051289	protein homotetramerization
GO:0070814	hydrogen sulfide biosynthetic process

Cellular Component

GO:0005634	nucleus
GO:0005737	cytoplasm
GO:0005829	cytosol
GO:0070062	extracellular vesicular exosome

Protein Structure and Domains

PDB ID

InterPro

IPR000277 Cys/Met metabolism, pyridoxal phosphate-dependent enzyme
IPR000653 DegT/DnrJ/EryC1/StrS aminotransferase
IPR015424 Pyridoxal phosphate-dependent transferase

PFAM

PF01053
PF01041

PRINTS

PIRSF

PIRSF001434
PIRSF000390

SMART

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

P32929