CTH - cystathionine gamma-lyase Gene

Also Known as CGL; CSE

Species: Homo sapiens

Gene Type: protein coding
Gene ID: 1491

About CTH

Cytogenetic location: 1p31.1 Genomic coordinates (GRCh38): 1:70,411,268-70,439,851 (from NCBI)

This gene has 5 transcripts (splice variants), 220 orthologues and is associated with 2 phenotypes. Biased expression in liver (RPKM 17.3), adrenal (RPKM 4.0) and 12 other tissues.

Summary

This gene encodes a cytoplasmic enzyme in the trans-sulfuration pathway that converts cystathione derived from methionine into cysteine. Glutathione synthesis in the liver is dependent upon the availability of cysteine. Mutations in this gene cause cystathioninuria. Alternative splicing of this gene results in three transcript variants encoding different isoforms. [provided by RefSeq, Jun 2010]

CTH Products (3)

mRNA Protein Name
NM_001190463.2 NP_001177392.1 cystathionine gamma-lyase isoform 3
NM_001902.6 NP_001893.2 cystathionine gamma-lyase isoform 1
NM_153742.5 NP_714964.2 cystathionine gamma-lyase isoform 2
Molecular Function GO Annotation Evidence References Source
enables L-cystine L-cysteine-lyase (deaminating) IMP
IMP: Inferred from mutant phenotype
22169477 GOA
enables cystathionine gamma-lyase activity IDA
IDA: Inferred from direct assay
10212249 GOA
enables identical protein binding IPI
IPI: Inferred from physical interaction
16189514 GOA
enables protein binding IPI
IPI: Inferred from physical interaction
22169477 GOA
enables pyridoxal phosphate binding IDA
IDA: Inferred from direct assay
10212249 GOA
Biological Process GO Annotation Evidence References Source
involved in cysteine biosynthetic process IDA
IDA: Inferred from direct assay
10212249 GOA
involved in cysteine biosynthetic process via cystathionine IDA
IDA: Inferred from direct assay
19428278 GOA
involved in hydrogen sulfide biosynthetic process IDA
IDA: Inferred from direct assay
19019829 GOA
involved in positive regulation of aortic smooth muscle cell differentiation IMP
IMP: Inferred from mutant phenotype
21659522 GOA
involved in protein homotetramerization IPI
IPI: Inferred from physical interaction
19019829 GOA
involved in protein sulfhydration IMP
IMP: Inferred from mutant phenotype
22169477 GOA
involved in protein-pyridoxal-5-phosphate linkage via peptidyl-N6-pyridoxal phosphate-L-lysine IDA
IDA: Inferred from direct assay
19019829 GOA
involved in transsulfuration IDA
IDA: Inferred from direct assay
19428278 GOA
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

CTH Protein Structure

Cys_Met_Meta_PP

Cys_Met_Meta_PP: Cys/Met metabolism PLP-dependent enzyme (19 - 395)

  • 0
  • 100
  • 200
  • 300
  • 405 a.a.
Protein Preferred Names Protein Names

cystathionine gamma-lyase

  • cystathionase (cystathionine gamma-lyase)

CTH Protein-protein interaction Information

Type
Protein Name Protein ID Interactor Interactor Species Interactor ID Detection Method References
Intra
CTH P32929 RECK Homo sapiens Q6P9E2 25416956
Intra
CTH P32929 RECK Homo sapiens Q6P9E2 32296183
Intra
CTH P32929 GUCD1 Homo sapiens Q96NT3-2 32296183
Intra
CTH P32929 NTAQ1 Homo sapiens Q96HA8 25416956
Intra
CTH P32929 NTAQ1 Homo sapiens Q96HA8 25416956
Intra
CTH P32929 CTH Homo sapiens P32929 16189514
Intra
CTH P32929 CTH Homo sapiens P32929 32296183
Intra
CTH P32929 CTH Homo sapiens P32929 32296183
Intra
CTH P32929 SDCBP2 Homo sapiens Q9H190 21988832
Intra
CTH P32929 CTH Homo sapiens P32929 32296183
Intra
CTH P32929 CTH Homo sapiens P32929 25416956
Intra
CTH P32929 GAPDHS Homo sapiens O14556 33961781
Intra
CTH P32929 CTH Homo sapiens P32929 32296183
Intra
CTH P32929 YWHAZ Homo sapiens P63104 15161933
Intra
CTH P32929 CTH Homo sapiens P32929 32296183
Intra
CTH P32929 GUCD1 Homo sapiens Q96NT3 25416956
Intra
CTH P32929 GUCD1 Homo sapiens Q96NT3 25416956
Cross: Cross-species interaction Intra: Intraspecies interaction

Recombinant CTH Proteins

Cat. No. Product Name Accession Purity
HY-P70025 Cystathionine gamma-lyase/CTH Protein, Human P32929 (M1-S405) ≥ 95%, as determined by reducing SDS-PAGE.

CTH Antibodies

Cat. No. Product Name Application Reactivity
HY-P82707 Cystathionase Antibody (YA2452) WB, IP Human

Related Diseases

Diseases Alias
Cystathioninuria
  • Cystathionase Deficiency

  • Gamma-Cystathionase Deficiency

  • Cystathione Gamma-Lyase Deficiency Syndrome

  • Cystathionine Gamma-Lyase Deficiency Syndrome

  • CSTNU

Hyperhomocysteinemia
Methionine Adenosyltransferase I/Iii Deficiency
  • Mat I/Iii Deficiency

  • Mat Deficiency

  • Methionine Adenosyltransferase Deficiency, Autosomal Recessive

  • Hypermethioninemia, Persistent, Autosomal Dominant, Due To Methionine Adenosyltransferase I/Iii Deficiency

  • Methionine Adenosyltransferase Deficiency

  • Hypermethioninemia, Isolated Persistent

  • Brain Demyelination Due To Methionine Adenosyltransferase Deficiency

  • MATD

  • Isolated Persistent Hypermethioninemia

  • Hepatic Methionine Adenosyltransferase Deficiency

  • Deficiency Of Acetyl-Coa Acetyltransferase

Encephalopathy, Ethylmalonic
  • Ethylmalonic Encephalopathy

  • EE

  • Epema Syndrome

  • Encephalopathy, Petechiae, And Ethylmalonic Aciduria

  • Ethe1 Deficiency

  • Eme

  • Syndrome Of Encephalopathy, Petechiae, And Ethylmalonic Aciduria

Hypermethioninemia
  • Hepatic Methionine Adenosyltransferase Deficiency

  • Deficiency Of Methionine Adenosyltransferase

  • Glycine N-Methyltransferase Deficiency

  • Met

  • S-Adenosylhomocysteine Hydrolase Deficiency

  • Gnmt Deficiency

  • Mat Deficiency

  • Methionine Adenosyltransferase Deficiency

  • Methioninemia

  • Deficiency Of Acetyl-Coa Acetyltransferase

Amino Acid Metabolic Disorder
  • Amino Acid Metabolism, Inborn Errors

  • Inborn Errors Of Amino Acid Metabolism

  • Disorder Of Amino Acid Metabolism

  • Amino Acid Metabolism Disorders

Hypertension, Essential
  • Essential Hypertension

  • Hypertension

  • High Blood Pressure

  • Hypertension, Essential, Susceptibility To

  • Hypertensive Disease

  • Primary Hypertension

  • EHT

  • Hypertension, Salt-Sensitive Essential, Susceptibility To

  • Hyperpiesia

  • Idiopathic Hypertension

  • Hypertensive Disorder

  • Hypertension, Essential, Susceptibility To, 3

  • Hypertension, Essential 3

  • Hypertension, Essential, Salt-Sensitive

  • Hypertension, Essential, Susceptibility To, 6

  • Hypertension, Essential 6

  • Hypertension, Salt-Sensitive Essential

  • Hypertension, Susceptibility To

  • Hypertension, Essential, Susceptibility To, 4

  • Hypertension, Essential 4

  • Hypertension, Essential, Susceptibility To, 2

  • Hypertension, Essential 2

  • Hypertension, Essential, Susceptibility To, 1

  • Hypertension, Essential 1

  • Hypertension, Essential, Susceptibility To, 5

  • Hypertension, Essential 5

  • Htn

  • Vascular Hypertensive Disorder

  • Systemic Primary Arterial Hypertension

  • Hbp - [High Blood Pressure]

  • Systemic Arterial Hypertensive Disorder

  • Elevated Blood Pressure

  • Arterial Hypertension Nos

  • Hypertension Nos

  • Benign Hypertension

  • Systemic Arterial Hypertension

  • Systemic Hypertension

  • Artery Htn

  • Benign Htn

  • Vascular Htn

  • Vascular Hypertension

  • Cholesterol Hypertension

  • Cholesterol Htn

  • Idiopathic Htn

  • Malignant Hypertension

  • Malignant Htn

  • Raised Blood Pressure

  • Cardiovascular Hypertension

  • Primary Htn - [Hypertension]

  • High Arterial Tension

  • High Blood Pressure Disorder

  • Ht - [Hypertension]

  • Htn - [Hypertension]

  • Hypertensive Vascular Disease

  • Hypertensive Vascular Degeneration

Diseases Alias
Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Orthologs Information

Species Symbol Source ID
Felis catus CTH VGNC VGNC:61250
Mus musculus CTH MGD MGI:1339968
Bos taurus CTH VGNC VGNC:27795
Rattus norvegicus CTH RGD RGD:2443
Canis familiaris CTH VGNC VGNC:39697
Macaca mulatta CTH VGNC VGNC:99872
Others CTH NCBI