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MT000022

UniProt Annotations

Entry Information

Gene Name	5'-aminolevulinate synthase 1
Protein Entry	HEM1_HUMAN
UniProt ID	P13196
Species	Human

Comments

Comment type	Description
Alternative Products	Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=P13196-1; Sequence=Displayed; Name=2; IsoId=P13196-2; Sequence=VSP_025925, VSP_025924; Note=No experimental confirmation available.;
Catalytic Activity	Succinyl-CoA + glycine = 5-aminolevulinate + CoA + CO(2).
Cofactor	Name=pyridoxal 5'-phosphate; Xref=ChEBI:CHEBI:597326;
Miscellaneous	There are two delta-ALA synthases in vertebrates: an erythroid- specific form and one (housekeeping) which is expressed in all tissues.
Pathway	Porphyrin-containing compound metabolism; protoporphyrin- IX biosynthesis; 5-aminolevulinate from glycine: step 1/1.
Sequence Caution	Sequence=CAA68506.1; Type=Frameshift; Positions=Several; Evidence={ECO:0000305};
Similarity	Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family. {ECO:0000305}.
Subcellular Location	Mitochondrion matrix.
Subunit	Homodimer.