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Protein

5-aminolevulinate synthase, erythroid-specific, mitochondrial

Gene

Alas2

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

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

Succinyl-CoA + glycine = 5-aminolevulinate + CoA + CO2.

Cofactori

Pathwayi: protoporphyrin-IX biosynthesis

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:
  1. 5-aminolevulinate synthase, nonspecific, mitochondrial (Alas1), 5-aminolevulinate synthase, erythroid-specific, mitochondrial (Alas2), 5-aminolevulinate synthase (Alas2), 5-aminolevulinate synthase (Alas2), 5-aminolevulinate synthase (Alas2), 5-aminolevulinate synthase (Alas1), 5-aminolevulinate synthase (Alas1), 5-aminolevulinate synthase (Alas2), 5-aminolevulinate synthase (Alas2)
This subpathway is part of the pathway protoporphyrin-IX biosynthesis, which is itself part of Porphyrin-containing compound metabolism.
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 keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei163SubstrateBy similarity1
Binding sitei280SubstrateBy similarity1
Binding sitei299SubstrateBy similarity1
Binding sitei332Pyridoxal phosphateBy similarity1
Binding sitei360Pyridoxal phosphateBy similarity1
Binding sitei388Pyridoxal phosphateBy similarity1
Active sitei391By similarity1
Binding sitei420Pyridoxal phosphate; shared with dimeric partnerBy similarity1
Binding sitei421Pyridoxal phosphate; shared with dimeric partnerBy similarity1
Binding sitei508SubstrateBy similarity1

GO - Molecular functioni

  • 5-aminolevulinate synthase activity Source: MGI
  • coenzyme binding Source: MGI
  • glycine binding Source: MGI
  • pyridoxal phosphate binding Source: InterPro

GO - Biological processi

Keywordsi

Molecular functionAcyltransferase, Transferase
Biological processHeme biosynthesis
LigandPyridoxal phosphate

Enzyme and pathway databases

ReactomeiR-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
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome X

Organism-specific databases

MGIiMGI:87990 Alas2

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi391K → A or H: Abolishes enzyme activity. 1

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 49Mitochondrion1 PublicationAdd BLAST49
ChainiPRO_000000122450 – 5875-aminolevulinate synthase, erythroid-specific, mitochondrialAdd BLAST538

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei391N6-(pyridoxal phosphate)lysine1 Publication1

Proteomic databases

MaxQBiP08680
PaxDbiP08680
PRIDEiP08680

PTM databases

PhosphoSitePlusiP08680

Expressioni

Tissue specificityi

Erythroid specific.

Gene expression databases

BgeeiENSMUSG00000025270 Expressed in 183 organ(s), highest expression level in blood
CleanExiMM_ALAS2
ExpressionAtlasiP08680 baseline and differential
GenevisibleiP08680 MM

Interactioni

Subunit structurei

Interacts with SUCLA2 (By similarity). Homodimer.By similarity

Protein-protein interaction databases

BioGridi198059, 1 interactor
IntActiP08680, 4 interactors
STRINGi10090.ENSMUSP00000066040

Structurei

Secondary structure

1587
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliP08680
SMRiP08680
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP08680

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1360 Eukaryota
COG0156 LUCA
GeneTreeiENSGT00530000063111
HOGENOMiHOG000221020
HOVERGENiHBG005954
InParanoidiP08680
KOiK00643
OrthoDBiEOG091G043U
PhylomeDBiP08680
TreeFamiTF300724

Family and domain databases

Gene3Di3.40.640.10, 1 hit
3.90.1150.10, 1 hit
InterProiView 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
PfamiView protein in Pfam
PF00155 Aminotran_1_2, 1 hit
PF09029 Preseq_ALAS, 1 hit
SUPFAMiSSF53383 SSF53383, 1 hit
TIGRFAMsiTIGR01821 5aminolev_synth, 1 hit
PROSITEiView protein in PROSITE
PS00599 AA_TRANSFER_CLASS_2, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All

P08680-1 [UniParc]FASTAAdd to basket
« Hide
        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
Length:587
Mass (Da):64,753
Last modified:January 23, 2002 - v3
Checksum:iA878D79FAD5B9856
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A2AFM1A2AFM1_MOUSE
5-aminolevulinate synthase
Alas2
572Annotation score:

Sequence cautioni

The sequence AAA37207 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti61 – 75Missing in AAA37207 (PubMed:3557128).CuratedAdd BLAST15
Sequence conflicti66A → R in BAB22254 (PubMed:16141072).Curated1
Sequence conflicti171W → R in AAA91866 (Ref. 2) Curated1
Sequence conflicti338C → V in AAA91866 (Ref. 2) Curated1
Sequence conflicti368 – 372RGAGI → GVQVS in AAA91866 (Ref. 2) Curated5
Sequence conflicti427V → M in AAA37207 (PubMed:3557128).Curated1
Sequence conflicti479R → W in AAA91866 (Ref. 2) Curated1

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
CCDSiCCDS41173.1
PIRiA29040 SYMSAL
RefSeqiNP_001095916.1, NM_001102446.1
NP_033783.1, NM_009653.3
UniGeneiMm.302724

Genome annotation databases

EnsembliENSMUST00000066337; ENSMUSP00000066040; ENSMUSG00000025270
GeneIDi11656
KEGGimmu:11656
UCSCiuc009uoj.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
CCDSiCCDS41173.1
PIRiA29040 SYMSAL
RefSeqiNP_001095916.1, NM_001102446.1
NP_033783.1, NM_009653.3
UniGeneiMm.302724

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1H7DNMR-A1-49[»]
1H7JNMR-A1-26[»]
ProteinModelPortaliP08680
SMRiP08680
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi198059, 1 interactor
IntActiP08680, 4 interactors
STRINGi10090.ENSMUSP00000066040

PTM databases

PhosphoSitePlusiP08680

Proteomic databases

MaxQBiP08680
PaxDbiP08680
PRIDEiP08680

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000066337; ENSMUSP00000066040; ENSMUSG00000025270
GeneIDi11656
KEGGimmu:11656
UCSCiuc009uoj.1 mouse

Organism-specific databases

CTDi212
MGIiMGI:87990 Alas2

Phylogenomic databases

eggNOGiKOG1360 Eukaryota
COG0156 LUCA
GeneTreeiENSGT00530000063111
HOGENOMiHOG000221020
HOVERGENiHBG005954
InParanoidiP08680
KOiK00643
OrthoDBiEOG091G043U
PhylomeDBiP08680
TreeFamiTF300724

Enzyme and pathway databases

UniPathwayi
UPA00251;UER00375

ReactomeiR-MMU-189451 Heme biosynthesis

Miscellaneous databases

EvolutionaryTraceiP08680
PROiPR:P08680
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000025270 Expressed in 183 organ(s), highest expression level in blood
CleanExiMM_ALAS2
ExpressionAtlasiP08680 baseline and differential
GenevisibleiP08680 MM

Family and domain databases

Gene3Di3.40.640.10, 1 hit
3.90.1150.10, 1 hit
InterProiView 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
PfamiView protein in Pfam
PF00155 Aminotran_1_2, 1 hit
PF09029 Preseq_ALAS, 1 hit
SUPFAMiSSF53383 SSF53383, 1 hit
TIGRFAMsiTIGR01821 5aminolev_synth, 1 hit
PROSITEiView protein in PROSITE
PS00599 AA_TRANSFER_CLASS_2, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiHEM0_MOUSE
AccessioniPrimary (citable) accession number: P08680
Secondary accession number(s): Q64452, Q9DCN0
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 1, 1988
Last sequence update: January 23, 2002
Last modified: November 7, 2018
This is version 169 of the entry and version 3 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  3. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  4. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
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Main funding by: National Institutes of Health

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