Names & Taxonomy
- Uniprot ID:
- P13196
- Entry Name:
- HEM1_HUMAN
- Status:
- reviewed
- Protein Names:
- 5-aminolevulinate synthase, nonspecific, mitochondrial (ALAS-H) (EC 2.3.1.37) (5-aminolevulinic acid synthase 1) (Delta-ALA synthase 1) (Delta-aminolevulinate synthase 1)
- Gene Names:
- ALAS1 ALAS3 ALASH OK/SW-cl.121
- Gene Names Orf:
- OK/SW-cl.121
- Gene Names Primary:
- ALAS1
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 640
- Sequence:
- MESVVRRCPFLSRVPQAFLQKAGKSLLFYAQNCPKMMEVGAKPAPRALSTAAVHYQQIKETPPASEKDKTAKAKVQQTPDGSQQSPDGTQLPSGHPLPATSQGTASKCPFLAAQMNQRGSSVFCKASLELQEDVQEMNAVRKEVAETSAGPSVVSVKTDGGDPSGLLKNFQDIMQKQRPERVSHLLQDNLPKSVSTFQYDRFFEKKIDEKKNDHTYRVFKTVNRRAHIFPMADDYSDSLITKKQVSVWCSNDYLGMSRHPRVCGAVMDTLKQHGAGAGGTRNISGTSKFHVDLERELADLHGKDAALLFSSCFVANDSTLFTLAKMMPGCEIYSDSGNHASMIQGIRNSRVPKYIFRHNDVSHLRELLQRSDPSVPKIVAFETVHSMDGAVCPLEELCDVAHEFGAITFVDEVHAVGLYGARGGGIGDRDGVMPKMDIISGTLGKAFGCVGGYIASTSSLIDTVRSYAAGFIFTTSLPPMLLAGALESVRILKSAEGRVLRRQHQRNVKLMRQMLMDAGLPVVHCPSHIIPVRVADAAKNTEVCDELMSRHNIYVQAINYPTVPRGEELLRIAPTPHHTPQMMNYFLENLLVTWKQVGLELKPHSSAECNFCRRPLHFEVMSEREKSYFSGLSKLVSAQA
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion matrix.
Function
- Pathway:
- Porphyrin-containing compound metabolism; protoporphyrin-IX biosynthesis; 5-aminolevulinate from glycine: step 1/1.
- Catalytic Activity:
- Succinyl-CoA + glycine = 5-aminolevulinate + CoA + CO(2).
- Cofactor:
- COFACTOR: Name=pyridoxal 5'-phosphate; Xref=ChEBI:CHEBI:597326;
- Active Site:
- ACT_SITE 445 445
- Cross Reference Drug Bank:
- DB00145
- Gene Ontology Go:
- cytoplasm
mitochondrial matrix
mitochondrion
nucleoplasm
5-aminolevulinate synthase activity
pyridoxal phosphate binding
cellular lipid metabolic process
heme biosynthetic process
mitochondrion organization
organelle organization
porphyrin-containing compound metabolic process
protoporphyrinogen IX biosynthetic process
small molecule metabolic process - Gene Ontology Biological Process:
- cellular lipid metabolic process
heme biosynthetic process
mitochondrion organization
organelle organization
porphyrin-containing compound metabolic process
protoporphyrinogen IX biosynthetic process
small molecule metabolic process - Gene Ontology Molecular Function:
- 5-aminolevulinate synthase activity
pyridoxal phosphate binding - Gene Ontology Cellular Component:
- cytoplasm
mitochondrial matrix
mitochondrion
nucleoplasm - Keywords:
- Acyltransferase
Alternative splicing
Complete proteome
Heme biosynthesis
Mitochondrion
Pyridoxal phosphate
Reference proteome
Transferase
Transit peptide - Interacts With:
- Q4VC05; Q9NWW7; Q8TD31-3; Q6P1J9; Q8TCT0; Q8WTR2; Q96L91; Q17RB0; Q9NVN8; Q6PF15; Q17RB8; Q6P444; Q9Y2S7; O60437; Q9H788; Q7Z614; Q15560; Q8WW24; P63313; Q0P5Q0; Q5W5X9; Q9Y2K6; O43516; Q96H43; Q9Y473; Q8TBZ8