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histidine ammonia-lyase; histidase (HAL, HIS)
Function:
- amino-acid degradation; L-histidine degradation into L-glutamate; N-formimidoyl-L-glutamate from L-histidine: step 1/3
- contains an active site 4-methylidene-imidazol-5-one (MIO), which is formed autocatalytically by cyclization & dehydration of residues Ala-Ser-Gly (putative)
L-histidine urocanate + NH3
Structure: belongs to the PAL/histidase family
Pathology:
- defects in HAL are the cause of histidinemia
General
phosphoprotein
protease; proteinase; endopeptidase
Properties
SIZE: entity length = 657 aa
MW = 73 kD
MOTIF: Ser phosphorylation site {S631}
Ser phosphorylation site {S635}
Ser phosphorylation site {S648}
Database Correlations
OMIM correlations
UniProt P42357
Pfam PF00221
Entrez Gene 3034
KEGG correlations
ENZYME 4.3.1.3
References
- UniProt :accession P42357
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=HAL