関: histidase

WordNet

a pungent gas compounded of nitrogen and hydrogen (NH3)
an essential amino acid found in proteins that is important for the growth and repair of tissue

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アンモニア / アンモニア水(ammonia water)

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/01/21 19:30:21」(JST)

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Histidine ammonia-lyase (or histidase, or histidinase) is an enzyme that in humans is encoded by the HAL gene.^[1]^[2] Histidase converts histidine into ammonia and urocanic acid.

Function

Histidine ammonia-lyase is a cytosolic enzyme catalyzing the first reaction in histidine catabolism, the nonoxidative deamination of L-histidine to trans-urocanic acid.^[1] The reaction is catalyzed by an electrophilic co-factor which is formed autocatalytically by cyclization of the protein backbone of the enzyme.^[3]

Pathology

Mutations in the gene for histidase are associated with histidinemia and urocanic aciduria.

References

^ ^a ^b "Entrez Gene: histidine ammonia-lyase".
^ Suchi M, Sano H, Mizuno H, Wada Y (September 1995). "Molecular cloning and structural characterization of the human histidase gene (HAL)". Genomics 29 (1): 98–104. doi:10.1006/geno.1995.1219. PMID 8530107.
^ Schwede, TF; Rétey, J; Schulz, GE (Apr 27, 1999). "Crystal structure of histidine ammonia-lyase revealing a novel polypeptide modification as the catalytic electrophile.". Biochemistry 38 (17): 5355–5361. doi:10.1021/bi982929q. PMID 10220322.

External links

Histidine Ammonia-Lyase at the US National Library of Medicine Medical Subject Headings (MeSH)

This article incorporates text from the United States National Library of Medicine, which is in the public domain.

This lyase article is a stub. You can help Wikipedia by expanding it.

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histidine ammonia-lyase
Identifiers
EC number	4.3.1.3
CAS number	9013-75-6
Databases
IntEnz	IntEnz view
BRENDA	BRENDA entry
ExPASy	NiceZyme view
KEGG	KEGG entry
MetaCyc	metabolic pathway
PRIAM	profile
PDB structures	RCSB PDB PDBe PDBsum
Gene Ontology	AmiGO / EGO
Search
PMC	articles
PubMed	articles
NCBI	proteins

Histidine ammonia-lyase
Identifiers
Symbols	HAL ; HIS; HSTD
External IDs	OMIM: 609457 MGI: 96010 HomoloGene: 68229 ChEMBL: 4003 GeneCards: HAL Gene
EC number	4.3.1.3
Gene ontology
Molecular function	• histidine ammonia-lyase activity;
Cellular component	• cytosol;
Biological process	• histidine catabolic process; • biosynthetic process; • histidine catabolic process to glutamate and formamide; • histidine catabolic process to glutamate and formate; • cellular nitrogen compound metabolic process; • small molecule metabolic process;
	Sources: Amigo / QuickGO
Orthologs
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