Entity Details

Primary name HEM0_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP22557
EntryNameHEM0_HUMAN
FullName5-aminolevulinate synthase, erythroid-specific, mitochondrial
TaxID9606
Evidenceevidence at protein level
Length587
SequenceStatuscomplete
DateCreated1991-08-01
DateModified2021-06-02

Ontological Relatives

GenesALAS2

GO terms

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GOName
GO:0001666 response to hypoxia
GO:0003870 5-aminolevulinate synthase activity
GO:0005739 mitochondrion
GO:0005743 mitochondrial inner membrane
GO:0005759 mitochondrial matrix
GO:0006782 protoporphyrinogen IX biosynthetic process
GO:0006783 heme biosynthetic process
GO:0006879 cellular iron ion homeostasis
GO:0030170 pyridoxal phosphate binding
GO:0030218 erythrocyte differentiation
GO:0032364 oxygen homeostasis
GO:0042541 hemoglobin biosynthetic process
GO:0048821 erythrocyte development

Subcellular Location

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Subcellular Location
Mitochondrion matrix

Domains

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DomainNameCategoryType
IPR001917 Aminotransferase, class-II, pyridoxal-phosphate binding siteSiteBinding site
IPR004839 Aminotransferase, class I/classIIDomainDomain
IPR010961 Tetrapyrrole biosynthesis, 5-aminolevulinic acid synthaseDomainDomain
IPR015118 5-aminolevulinate synthase presequenceDomainDomain
IPR015421 Pyridoxal phosphate-dependent transferase, major domainFamilyHomologous superfamily
IPR015422 Pyridoxal phosphate-dependent transferase, small domainFamilyHomologous superfamily
IPR015424 Pyridoxal phosphate-dependent transferaseFamilyHomologous superfamily

Diseases

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Drugs

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DrugNameSourceType
DB00114 Pyridoxal phosphateDrugbanksmall molecule
DB00145 GlycineDrugbanksmall molecule

Interactions

4 interactions