Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

4.4.1.1

Official Name

cystathionine gamma-lyase

Name from literature

cystathionine-gamma-lyase

Pathway from literature

the synthesis of cysteine from methionine

Pathway from KEGG

Amino Acid Metabolism; Glycine, serine and threonine metabolism; map00260

Metabolism of Other Amino Acids; Selenoamino acid metabolism; map00450

Amino Acid Metabolism; Methionine metabolism; map00271

Energy Metabolism; Nitrogen metabolism; map00910

Amino Acid Metabolism; Cysteine metabolism; map00272

Organisms

Human (9606)

Genome localization

1p31.1[1491 ],

Comments

A multifunctional pyridoxal-phosphate protein. Also catalyses elimination reactions of L-homoserine to form H2O, NH3 and 2-oxobutanoate, of L-cystine, producing thiocysteine, pyruvate and NH3, and of L-cysteine producing pyruvate, NH3 and H2S.

Rate-limiting Description

"Cystathionine gamma-lyase is the rate-limiting enzyme for the synthesis of cysteine from methionine and availability of cysteine is a critical factor in glutathione synthesis." (17095121)

Regulatory Information

Regulatory type

Detail

phosphorylation;

P32929:from_uniprot:282_Phosphoserine

phosphorylation;

P32929:from_uniprot:377_Phosphoserine

phosphorylation;

P32929:from_uniprot:8_Phosphoserine

phosphorylation;

P32929

Gene ontology

Gene ontology

GO:0004123 (F) cystathionine gamma-lyase activity [P32929 ];
GO:0030170 (F) pyridoxal phosphate binding [P32929 ];
GO:0005737 (C) cytoplasm [P32929 ];
GO:0019344 (P) cysteine biosynthetic process [P32929 ];

Subcellular localization

Localization

cytoplasm [P32929 ];

Disease relevance

Disease

Defects in CTH are the cause of cystathioninuria [MIM:219500]. CTH is an autosomal recessive phenotype characterized by abnormal accumulation of plasma cystathionine, leading to increased urinary excretion [P32929 ];

Links

SwissProt

P32929

Entrez Gene

1491

HPRD

09633



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  Last Modified: 2009-03-24  
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