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

Publication

PubMed ID:
2263504 3671094 15489334