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Protein

5-aminolevulinate synthase, nonspecific, mitochondrial

Gene

Alas1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Experimental evidence at protein leveli

Functioni

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 (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)
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 sitei219SubstrateBy similarity1
Binding sitei336SubstrateBy similarity1
Binding sitei355SubstrateBy similarity1
Binding sitei388Pyridoxal phosphateBy similarity1
Binding sitei416Pyridoxal phosphateBy similarity1
Binding sitei444Pyridoxal phosphateBy similarity1
Active sitei447By similarity1
Binding sitei476Pyridoxal phosphate; shared with dimeric partnerBy similarity1
Binding sitei477Pyridoxal phosphate; shared with dimeric partnerBy similarity1
Binding sitei564SubstrateBy similarity1

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Acyltransferase, Transferase

Keywords - Biological processi

Heme biosynthesis

Keywords - Ligandi

Pyridoxal phosphate

Enzyme and pathway databases

ReactomeiR-MMU-189451. Heme biosynthesis.
UniPathwayiUPA00251; UER00375.

Names & Taxonomyi

Protein namesi
Recommended name:
5-aminolevulinate synthase, nonspecific, mitochondrial (EC:2.3.1.37)
Short name:
ALAS-H
Alternative name(s):
5-aminolevulinic acid synthase 1
Delta-ALA synthase 1
Delta-aminolevulinate synthase 1
Gene namesi
Name:Alas1
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 9

Organism-specific databases

MGIiMGI:87989. Alas1.

Subcellular locationi

GO - Cellular componenti

  • cytoplasm Source: MGI
  • mitochondrial matrix Source: UniProtKB-SubCell
  • mitochondrion Source: MGI
  • nucleoplasm Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 56MitochondrionBy similarityAdd BLAST56
ChainiPRO_000000123157 – 6425-aminolevulinate synthase, nonspecific, mitochondrialAdd BLAST586

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei447N6-(pyridoxal phosphate)lysineBy similarity1

Proteomic databases

MaxQBiQ8VC19.
PaxDbiQ8VC19.
PRIDEiQ8VC19.

PTM databases

iPTMnetiQ8VC19.
PhosphoSitePlusiQ8VC19.

Expressioni

Gene expression databases

BgeeiENSMUSG00000032786.
CleanExiMM_ALAS1.
ExpressionAtlasiQ8VC19. baseline and differential.
GenevisibleiQ8VC19. MM.

Interactioni

Subunit structurei

Homodimer.By similarity

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000108143.

Structurei

3D structure databases

ProteinModelPortaliQ8VC19.
SMRiQ8VC19.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1360. Eukaryota.
COG0156. LUCA.
GeneTreeiENSGT00530000063111.
HOGENOMiHOG000221020.
HOVERGENiHBG005954.
InParanoidiQ8VC19.
KOiK00643.
OMAiPQMMSYF.
OrthoDBiEOG091G043U.
PhylomeDBiQ8VC19.
TreeFamiTF300724.

Family and domain databases

Gene3Di3.40.640.10. 1 hit.
3.90.1150.10. 1 hit.
InterProiIPR010961. 4pyrrol_synth_NH2levulA_synth.
IPR015118. 5aminolev_synth_preseq.
IPR001917. Aminotrans_II_pyridoxalP_BS.
IPR004839. Aminotransferase_I/II.
IPR015424. PyrdxlP-dep_Trfase.
IPR015421. PyrdxlP-dep_Trfase_major_sub1.
IPR015422. PyrdxlP-dep_Trfase_major_sub2.
[Graphical view]
PfamiPF00155. Aminotran_1_2. 1 hit.
PF09029. Preseq_ALAS. 1 hit.
[Graphical view]
SUPFAMiSSF53383. SSF53383. 1 hit.
TIGRFAMsiTIGR01821. 5aminolev_synth. 1 hit.
PROSITEiPS00599. AA_TRANSFER_CLASS_2. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

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

Q8VC19-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
METVVRRCPF LSRVPQAFLQ KAGKSLLFYA QNCPKMMEVG AKPAPRTLST
60 70 80 90 100
SAVHCQQVKE TPPANEKEKT AKAAVQQAPD ESQMAQTPDG TQLPSGHPSP
110 120 130 140 150
ATSQGSGSKC PFLAAQLSQT GSSVFRKASL ELQEDVQEMH AVRKEAAQSP
160 170 180 190 200
VPPSLVNVKT DGEDPSRLLK NFQDIMRKQR PERVSHLLQD NLPKSVSTFQ
210 220 230 240 250
YDHFFEKKID EKKNDHTYRV FKTVNRRAQI FPMADDYTDS LITKKQVSVW
260 270 280 290 300
CSNDYLGMSR HPRVCGAVME TVKQHGAGAG GTRNISGTSK FHVELEQALA
310 320 330 340 350
DLHGKDAALL FSSCFVANDS TLFTLAKMMP GCEIYSDSGN HASMIQGIRN
360 370 380 390 400
SRVPKYIFRH NDVNHLRELL QRSDPSVPKI VAFETVHSMD GAVCPLEELC
410 420 430 440 450
DVAHEFGAIT FVDEVHAVGL YGARGGGIGD RDGVMPKMDI ISGTLGKAFG
460 470 480 490 500
CVGGYIASTS LLIDTVRSYA AGFIFTTSLP PMLLAGALES VRILKSSEGR
510 520 530 540 550
ALRRQHQRNV KLLRQMLMDA GLPVIHCPSH IIPVRVADAA KNTEICDELM
560 570 580 590 600
TRHNIYVQAI NYPTVPRGEE LLRIAPTPHH TPQMMNFFVE KLLVTWKRVG
610 620 630 640
LELKPHSSAE CNFCRRPLHF EVMSEREKAY FSGMSKMVSA QA
Length:642
Mass (Da):71,018
Last modified:September 27, 2004 - v2
Checksum:i34E0CBEB8599CFE5
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti192L → S in AAA91867 (Ref. 2) Curated1
Sequence conflicti391Missing in AAH22110 (PubMed:15489334).Curated1
Sequence conflicti463I → T in AAA91867 (Ref. 2) Curated1
Sequence conflicti501A → P in AAA91867 (Ref. 2) Curated1
Sequence conflicti602E → V in AAA91867 (Ref. 2) Curated1
Sequence conflicti625E → V in AAA91867 (Ref. 2) Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
BC022110 mRNA. Translation: AAH22110.1.
M63245 mRNA. Translation: AAA91867.1.
CCDSiCCDS40756.2.
RefSeqiNP_001278764.1. NM_001291835.1.
NP_065584.2. NM_020559.2.
UniGeneiMm.290578.

Genome annotation databases

EnsembliENSMUST00000112524; ENSMUSP00000108143; ENSMUSG00000032786.
ENSMUST00000141118; ENSMUSP00000117014; ENSMUSG00000032786.
GeneIDi11655.
KEGGimmu:11655.
UCSCiuc009rjj.2. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
BC022110 mRNA. Translation: AAH22110.1.
M63245 mRNA. Translation: AAA91867.1.
CCDSiCCDS40756.2.
RefSeqiNP_001278764.1. NM_001291835.1.
NP_065584.2. NM_020559.2.
UniGeneiMm.290578.

3D structure databases

ProteinModelPortaliQ8VC19.
SMRiQ8VC19.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000108143.

PTM databases

iPTMnetiQ8VC19.
PhosphoSitePlusiQ8VC19.

Proteomic databases

MaxQBiQ8VC19.
PaxDbiQ8VC19.
PRIDEiQ8VC19.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000112524; ENSMUSP00000108143; ENSMUSG00000032786.
ENSMUST00000141118; ENSMUSP00000117014; ENSMUSG00000032786.
GeneIDi11655.
KEGGimmu:11655.
UCSCiuc009rjj.2. mouse.

Organism-specific databases

CTDi211.
MGIiMGI:87989. Alas1.

Phylogenomic databases

eggNOGiKOG1360. Eukaryota.
COG0156. LUCA.
GeneTreeiENSGT00530000063111.
HOGENOMiHOG000221020.
HOVERGENiHBG005954.
InParanoidiQ8VC19.
KOiK00643.
OMAiPQMMSYF.
OrthoDBiEOG091G043U.
PhylomeDBiQ8VC19.
TreeFamiTF300724.

Enzyme and pathway databases

UniPathwayiUPA00251; UER00375.
ReactomeiR-MMU-189451. Heme biosynthesis.

Miscellaneous databases

ChiTaRSiAlas1. mouse.
PROiQ8VC19.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000032786.
CleanExiMM_ALAS1.
ExpressionAtlasiQ8VC19. baseline and differential.
GenevisibleiQ8VC19. MM.

Family and domain databases

Gene3Di3.40.640.10. 1 hit.
3.90.1150.10. 1 hit.
InterProiIPR010961. 4pyrrol_synth_NH2levulA_synth.
IPR015118. 5aminolev_synth_preseq.
IPR001917. Aminotrans_II_pyridoxalP_BS.
IPR004839. Aminotransferase_I/II.
IPR015424. PyrdxlP-dep_Trfase.
IPR015421. PyrdxlP-dep_Trfase_major_sub1.
IPR015422. PyrdxlP-dep_Trfase_major_sub2.
[Graphical view]
PfamiPF00155. Aminotran_1_2. 1 hit.
PF09029. Preseq_ALAS. 1 hit.
[Graphical view]
SUPFAMiSSF53383. SSF53383. 1 hit.
TIGRFAMsiTIGR01821. 5aminolev_synth. 1 hit.
PROSITEiPS00599. AA_TRANSFER_CLASS_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiHEM1_MOUSE
AccessioniPrimary (citable) accession number: Q8VC19
Secondary accession number(s): Q64453
Entry historyi
Integrated into UniProtKB/Swiss-Prot: September 27, 2004
Last sequence update: September 27, 2004
Last modified: November 2, 2016
This is version 124 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Miscellaneous

There are two delta-ALA synthases in vertebrates: an erythroid- specific form and one (housekeeping) which is expressed in all tissues.

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.