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Protein

5-aminolevulinate synthase, nonspecific, mitochondrial

Gene

Alas1

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 (Alas2), 5-aminolevulinate synthase (Alas2), 5-aminolevulinate synthase (Alas1), 5-aminolevulinate synthase (Alas2), 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

Keywordsi

Molecular functionAcyltransferase, Transferase
Biological processHeme biosynthesis
LigandPyridoxal 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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 9

Organism-specific databases

MGIiMGI:87989 Alas1

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

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
SwissPalmiQ8VC19

Expressioni

Gene expression databases

BgeeiENSMUSG00000032786
CleanExiMM_ALAS1
ExpressionAtlasiQ8VC19 baseline and differential
GenevisibleiQ8VC19 MM

Interactioni

Subunit structurei

Homodimer.By similarity

GO - Molecular functioni

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, 2 hits
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

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

Similar proteinsi

Entry informationi

Entry nameiHEM1_MOUSE
AccessioniPrimary (citable) accession number: Q8VC19
Secondary accession number(s): Q64453
Entry historyiIntegrated into UniProtKB/Swiss-Prot: September 27, 2004
Last sequence update: September 27, 2004
Last modified: May 23, 2018
This is version 130 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

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