UniProtKB - P08680 (HEM0_MOUSE)
Protein
5-aminolevulinate synthase, erythroid-specific, mitochondrial
Gene
Alas2
Organism
Mus musculus (Mouse)
Status
Functioni
Miscellaneous
There are two delta-ALA synthases in vertebrates: an erythroid- specific form and one (housekeeping) which is expressed in all tissues.
Catalytic activityi
- EC:2.3.1.37
Cofactori
: protoporphyrin-IX biosynthesis Pathwayi
This protein is involved in step 1 of the subpathway that synthesizes 5-aminolevulinate from glycine.Proteins known to be involved in this subpathway in this organism are:
- 5-aminolevulinate synthase (Alas2), 5-aminolevulinate synthase (Alas2), 5-aminolevulinate synthase (Alas2), 5-aminolevulinate synthase (Alas1), 5-aminolevulinate synthase, erythroid-specific, mitochondrial (Alas2), 5-aminolevulinate synthase, nonspecific, mitochondrial (Alas1), 5-aminolevulinate synthase (Alas2), 5-aminolevulinate synthase (Alas2), 5-aminolevulinate synthase (Alas1)
View all proteins of this organism that are known to be involved in the subpathway that synthesizes 5-aminolevulinate from glycine, the pathway protoporphyrin-IX biosynthesis and in Porphyrin-containing compound metabolism.
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Binding sitei | 163 | SubstrateBy similarity | 1 | |
Binding sitei | 280 | SubstrateBy similarity | 1 | |
Binding sitei | 299 | SubstrateBy similarity | 1 | |
Binding sitei | 332 | Pyridoxal phosphateBy similarity | 1 | |
Binding sitei | 360 | Pyridoxal phosphateBy similarity | 1 | |
Binding sitei | 388 | Pyridoxal phosphateBy similarity | 1 | |
Active sitei | 391 | By similarity | 1 | |
Binding sitei | 420 | Pyridoxal phosphate; shared with dimeric partnerBy similarity | 1 | |
Binding sitei | 421 | Pyridoxal phosphate; shared with dimeric partnerBy similarity | 1 | |
Binding sitei | 508 | SubstrateBy similarity | 1 |
GO - Molecular functioni
- 5-aminolevulinate synthase activity Source: MGI
- coenzyme A binding Source: MGI
- pyridoxal phosphate binding Source: InterPro
GO - Biological processi
- cellular iron ion homeostasis Source: MGI
- erythrocyte development Source: GO_Central
- erythrocyte differentiation Source: UniProtKB
- heme biosynthetic process Source: UniProtKB
- hemoglobin biosynthetic process Source: UniProtKB
- protoporphyrinogen IX biosynthetic process Source: UniProtKB-UniPathway
- response to hypoxia Source: UniProtKB
Keywordsi
Molecular function | Acyltransferase, Transferase |
Biological process | Heme biosynthesis |
Ligand | Pyridoxal phosphate |
Enzyme and pathway databases
Reactomei | R-MMU-189451, Heme biosynthesis |
UniPathwayi | UPA00251;UER00375 |
Names & Taxonomyi
Protein namesi | Recommended name: 5-aminolevulinate synthase, erythroid-specific, mitochondrial (EC:2.3.1.37)Short name: ALAS-E Alternative name(s): 5-aminolevulinic acid synthase 2 Delta-ALA synthase 2 Delta-aminolevulinate synthase 2 |
Gene namesi | Name:Alas2 |
Organismi | Mus musculus (Mouse) |
Taxonomic identifieri | 10090 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Myomorpha › Muroidea › Muridae › Murinae › Mus › Mus |
Proteomesi |
|
Organism-specific databases
MGIi | MGI:87990, Alas2 |
Subcellular locationi
Mitochondrion
Mitochondrion
- mitochondrial inner membrane Source: UniProtKB
- mitochondrial matrix Source: UniProtKB-SubCell
- mitochondrion Source: MGI
Keywords - Cellular componenti
MitochondrionPathology & Biotechi
Mutagenesis
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Mutagenesisi | 391 | K → A or H: Abolishes enzyme activity. | 1 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transit peptidei | 1 – 49 | Mitochondrion1 PublicationAdd BLAST | 49 | |
ChainiPRO_0000001224 | 50 – 587 | 5-aminolevulinate synthase, erythroid-specific, mitochondrialAdd BLAST | 538 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 391 | N6-(pyridoxal phosphate)lysine1 Publication | 1 |
Proteomic databases
MaxQBi | P08680 |
PaxDbi | P08680 |
PRIDEi | P08680 |
PTM databases
iPTMneti | P08680 |
PhosphoSitePlusi | P08680 |
Expressioni
Tissue specificityi
Erythroid specific.
Gene expression databases
Bgeei | ENSMUSG00000025270, Expressed in blood and 205 other tissues |
ExpressionAtlasi | P08680, baseline and differential |
Genevisiblei | P08680, MM |
Interactioni
Subunit structurei
Interacts with SUCLA2 (By similarity). Homodimer.
By similarityProtein-protein interaction databases
BioGRIDi | 198059, 3 interactors |
IntActi | P08680, 4 interactors |
STRINGi | 10090.ENSMUSP00000066040 |
Miscellaneous databases
RNActi | P08680, protein |
Structurei
Secondary structure
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details3D structure databases
SMRi | P08680 |
ModBasei | Search... |
PDBe-KBi | Search... |
Miscellaneous databases
EvolutionaryTracei | P08680 |
Family & Domainsi
Sequence similaritiesi
Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family.Curated
Keywords - Domaini
Transit peptidePhylogenomic databases
eggNOGi | KOG1360, Eukaryota |
GeneTreei | ENSGT00940000159912 |
InParanoidi | P08680 |
OrthoDBi | 930001at2759 |
PhylomeDBi | P08680 |
TreeFami | TF300724 |
Family and domain databases
Gene3Di | 3.40.640.10, 1 hit 3.90.1150.10, 1 hit |
InterProi | View protein in InterPro IPR010961, 4pyrrol_synth_NH2levulA_synth IPR015118, 5aminolev_synth_preseq IPR001917, Aminotrans_II_pyridoxalP_BS IPR004839, Aminotransferase_I/II IPR015424, PyrdxlP-dep_Trfase IPR015422, PyrdxlP-dep_Trfase_dom1 IPR015421, PyrdxlP-dep_Trfase_major |
Pfami | View protein in Pfam PF00155, Aminotran_1_2, 1 hit PF09029, Preseq_ALAS, 1 hit |
SUPFAMi | SSF53383, SSF53383, 1 hit |
TIGRFAMsi | TIGR01821, 5aminolev_synth, 1 hit |
PROSITEi | View protein in PROSITE PS00599, AA_TRANSFER_CLASS_2, 1 hit |
(1+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All
P08680-1 [UniParc]FASTAAdd to basket
10 20 30 40 50
MVAAAMLLRS CPVLSQGPTG LLGKVAKTYQ FLFSIGRCPI LATQGPTCSQ
60 70 80 90 100
IHLKATKAGG DSPSWAKSHC PFMLSELQDR KSKIVQRAAP EVQEDVKTFK
110 120 130 140 150
TDLLSTMDST TRSHSFPSFQ EPEQTEGAVP HLIQNNMTGS QAFGYDQFFR
160 170 180 190 200
DKIMEKKQDH TYRVFKTVNR WANAYPFAQH FSEASMASKD VSVWCSNDYL
210 220 230 240 250
GISRHPRVLQ AIEETLKNHG AGAGGTRNIS GTSKFHVELE QELAELHQKD
260 270 280 290 300
SALLFSSCFV ANDSTLFTLA KLLPGCEIYS DAGNHASMIQ GIRNSGAAKF
310 320 330 340 350
VFRHNDPGHL KKLLEKSDPK TPKIVAFETV HSMDGAICPL EELCDVAHQY
360 370 380 390 400
GALTFVDEVH AVGLYGARGA GIGERDGIMH KLDIISGTLG KAFGCVGGYI
410 420 430 440 450
ASTRDLVDMV RSYAAGFIFT TSLPPMVLSG ALESVRLLKG EEGQALRRAH
460 470 480 490 500
QRNVKHMRQL LMDRGFPVIP CPSHIIPIRV GNAALNSKIC DLLLSKHSIY
510 520 530 540 550
VQAINYPTVP RGEELLRLAP SPHHSPQMME NFVEKLLLAW TEVGLPLQDV
560 570 580
SVAACNFCHR PVHFELMSEW ERSYFGNMGP QYVTTYA
Computationally mapped potential isoform sequencesi
There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basketA2AFM1 | A2AFM1_MOUSE | 5-aminolevulinate synthase | Alas2 | 572 | Annotation score: |
Sequence cautioni
The sequence AAA37207 differs from that shown. Reason: Erroneous initiation.Curated
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 61 – 75 | Missing in AAA37207 (PubMed:3557128).CuratedAdd BLAST | 15 | |
Sequence conflicti | 66 | A → R in BAB22254 (PubMed:16141072).Curated | 1 | |
Sequence conflicti | 171 | W → R in AAA91866 (Ref. 2) Curated | 1 | |
Sequence conflicti | 338 | C → V in AAA91866 (Ref. 2) Curated | 1 | |
Sequence conflicti | 368 – 372 | RGAGI → GVQVS in AAA91866 (Ref. 2) Curated | 5 | |
Sequence conflicti | 427 | V → M in AAA37207 (PubMed:3557128).Curated | 1 | |
Sequence conflicti | 479 | R → W in AAA91866 (Ref. 2) Curated | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | M15268 mRNA Translation: AAA37207.1 Different initiation. M63244 mRNA Translation: AAA91866.1 AK002642 mRNA Translation: BAB22254.1 AK077610 mRNA Translation: BAC36898.1 |
CCDSi | CCDS41173.1 |
PIRi | A29040, SYMSAL |
RefSeqi | NP_001095916.1, NM_001102446.1 NP_033783.1, NM_009653.3 |
Genome annotation databases
Ensembli | ENSMUST00000066337; ENSMUSP00000066040; ENSMUSG00000025270 |
GeneIDi | 11656 |
KEGGi | mmu:11656 |
UCSCi | uc009uoj.1, mouse |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | M15268 mRNA Translation: AAA37207.1 Different initiation. M63244 mRNA Translation: AAA91866.1 AK002642 mRNA Translation: BAB22254.1 AK077610 mRNA Translation: BAC36898.1 |
CCDSi | CCDS41173.1 |
PIRi | A29040, SYMSAL |
RefSeqi | NP_001095916.1, NM_001102446.1 NP_033783.1, NM_009653.3 |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
1H7D | NMR | - | A | 1-49 | [»] | |
1H7J | NMR | - | A | 1-26 | [»] | |
SMRi | P08680 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 198059, 3 interactors |
IntActi | P08680, 4 interactors |
STRINGi | 10090.ENSMUSP00000066040 |
PTM databases
iPTMneti | P08680 |
PhosphoSitePlusi | P08680 |
Proteomic databases
MaxQBi | P08680 |
PaxDbi | P08680 |
PRIDEi | P08680 |
Protocols and materials databases
Antibodypediai | 457, 294 antibodies |
Genome annotation databases
Ensembli | ENSMUST00000066337; ENSMUSP00000066040; ENSMUSG00000025270 |
GeneIDi | 11656 |
KEGGi | mmu:11656 |
UCSCi | uc009uoj.1, mouse |
Organism-specific databases
CTDi | 212 |
MGIi | MGI:87990, Alas2 |
Phylogenomic databases
eggNOGi | KOG1360, Eukaryota |
GeneTreei | ENSGT00940000159912 |
InParanoidi | P08680 |
OrthoDBi | 930001at2759 |
PhylomeDBi | P08680 |
TreeFami | TF300724 |
Enzyme and pathway databases
UniPathwayi | UPA00251;UER00375 |
Reactomei | R-MMU-189451, Heme biosynthesis |
Miscellaneous databases
BioGRID-ORCSi | 11656, 0 hits in 17 CRISPR screens |
EvolutionaryTracei | P08680 |
PROi | PR:P08680 |
RNActi | P08680, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSMUSG00000025270, Expressed in blood and 205 other tissues |
ExpressionAtlasi | P08680, baseline and differential |
Genevisiblei | P08680, MM |
Family and domain databases
Gene3Di | 3.40.640.10, 1 hit 3.90.1150.10, 1 hit |
InterProi | View protein in InterPro IPR010961, 4pyrrol_synth_NH2levulA_synth IPR015118, 5aminolev_synth_preseq IPR001917, Aminotrans_II_pyridoxalP_BS IPR004839, Aminotransferase_I/II IPR015424, PyrdxlP-dep_Trfase IPR015422, PyrdxlP-dep_Trfase_dom1 IPR015421, PyrdxlP-dep_Trfase_major |
Pfami | View protein in Pfam PF00155, Aminotran_1_2, 1 hit PF09029, Preseq_ALAS, 1 hit |
SUPFAMi | SSF53383, SSF53383, 1 hit |
TIGRFAMsi | TIGR01821, 5aminolev_synth, 1 hit |
PROSITEi | View protein in PROSITE PS00599, AA_TRANSFER_CLASS_2, 1 hit |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | HEM0_MOUSE | |
Accessioni | P08680Primary (citable) accession number: P08680 Secondary accession number(s): Q64452, Q9DCN0 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | January 1, 1988 |
Last sequence update: | January 23, 2002 | |
Last modified: | December 2, 2020 | |
This is version 182 of the entry and version 3 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program |
Miscellaneousi
Keywords - Technical termi
3D-structure, Reference proteomeDocuments
- MGD cross-references
Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot - PATHWAY comments
Index of metabolic and biosynthesis pathways - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families