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S-adenosyl homocysteine hydrolase (ADOHCYASE, adenosylhomocysteinase, AHCY, SAHH)
Function:
- hydrolyzes S-adenosyl-5'-homocysteine to homocysteine & adenosine, a thioether cleavage
- adenosylhomocysteine is a competitive inhibitor of S-adenosylmethionine-dependent methyl transferase reactions, thus AHCY may play a role in control of methylations via regulation of intracellular concentration of adenosylhomocysteine
- amino-acid biosynthesis, homocysteine biosynthesis
- L-homocysteine from S-adenosyl-L-homocysteine: step 1/1
- context: activated methyl cycle
S-adenosyl-L-homocysteine + H2O L-homocysteine + adenosine
Cofactor: binds 1 NAD per subunit
Structure:
- homotetramer
- belongs to the adenosylhomocysteinase family
Compartment:
- cytoplasm, melanosome
Pathology:
- defects in AHCY are a cause of hypermethioninemia
Genetics: Gene locus: 20cen-q13.1
Laboratory:
- adenosylhomocysteine hydrolase in erythrocytes
Interactions
molecular events
Related
adenosine (Adenocard, Adenoscan)
adenosylhomocysteinase 2; AdoHcyase 2; DC-expressed AHCY-like molecule; S-adenosyl-L-homocysteine hydrolase 2; S-adenosylhomocysteine hydrolase-like protein 1 (AHCYL1, DCAL, XPVKONA)
adenosylhomocysteinase 3; S-adenosyl-L-homocysteine hydrolase 3; AdoHcyase 3 (AHCYL2, KIAA0828)
AHCY gene mutation
homocysteine
methionine metabolism (trans-sulfuration pathway)
S-adenosylhomocysteine
General
hydrolase
Properties
SIZE: entity length = 432 aa
MW = 48 kD
COMPARTMENT: cytoplasm
MOTIF: binding site
SITE: 57-57
FOR-BINDING-OF: Substrate
binding site
SITE: 131-131
FOR-BINDING-OF: Substrate
binding site
SITE: 156-156
FOR-BINDING-OF: Substrate
NAD binding {183-350}
MOTIF: binding site
SITE: 186-186
FOR-BINDING-OF: Substrate
binding site
SITE: 190-190
FOR-BINDING-OF: Substrate
Database Correlations
OMIM 180960
MORBIDMAP 180960
UniProt P23526
Entrez Gene 191
KEGG correlations
ENZYME 3.3.1.1
References
- OMIM :accession 180960
- Stryer Biochemistry WH Freeman & Co, New York,
1988, pg 583