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Q8VC19

- HEM1_MOUSE

UniProt

Q8VC19 - HEM1_MOUSE

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Protein

5-aminolevulinate synthase, nonspecific, mitochondrial

Gene
Alas1
Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 4 out of 5 - Experimental evidence at transcript leveli

Functioni

Catalytic activityi

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

Cofactori

Pyridoxal phosphate By similarity.

Pathwayi

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei219 – 2191Substrate By similarity
Binding sitei336 – 3361Substrate By similarity
Binding sitei355 – 3551Substrate By similarity
Binding sitei388 – 3881Pyridoxal phosphate By similarity
Binding sitei416 – 4161Pyridoxal phosphate By similarity
Binding sitei444 – 4441Pyridoxal phosphate By similarity
Active sitei447 – 4471 By similarity
Binding sitei476 – 4761Pyridoxal phosphate By similarity
Binding sitei477 – 4771Pyridoxal phosphate By similarity
Binding sitei564 – 5641Substrate By similarity

GO - Molecular functioni

  1. 5-aminolevulinate synthase activity Source: UniProtKB-EC
  2. pyridoxal phosphate binding Source: InterPro

GO - Biological processi

  1. protoporphyrinogen IX biosynthetic process Source: UniProtKB-UniPathway
Complete GO annotation...

Keywords - Molecular functioni

Acyltransferase, Transferase

Keywords - Biological processi

Heme biosynthesis

Keywords - Ligandi

Pyridoxal phosphate

Enzyme and pathway databases

ReactomeiREACT_198602. PPARA activates gene expression.
REACT_203298. Heme biosynthesis.
REACT_205251. Transcriptional activation of mitochondrial biogenesis.
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
ProteomesiUP000000589: Chromosome 9

Organism-specific databases

MGIiMGI:87989. Alas1.

Subcellular locationi

Mitochondrion matrix By similarity

GO - Cellular componenti

  1. mitochondrial matrix Source: UniProtKB-SubCell
  2. mitochondrion Source: MGI
  3. nucleus Source: Ensembl
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 5656Mitochondrion By similarityAdd
BLAST
Chaini57 – 6425865-aminolevulinate synthase, nonspecific, mitochondrialPRO_0000001231Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei447 – 4471N6-(pyridoxal phosphate)lysine By similarity

Proteomic databases

MaxQBiQ8VC19.
PaxDbiQ8VC19.
PRIDEiQ8VC19.

PTM databases

PhosphoSiteiQ8VC19.

Expressioni

Gene expression databases

ArrayExpressiQ8VC19.
BgeeiQ8VC19.
CleanExiMM_ALAS1.
GenevestigatoriQ8VC19.

Interactioni

Subunit structurei

Homodimer By similarity.

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000117014.

Structurei

3D structure databases

ProteinModelPortaliQ8VC19.
SMRiQ8VC19. Positions 201-592.

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG0156.
GeneTreeiENSGT00530000063111.
HOGENOMiHOG000221020.
HOVERGENiHBG005954.
InParanoidiQ8VC19.
KOiK00643.
OMAiPQMMSYF.
OrthoDBiEOG7TQV0F.
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. 2 hits.
[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

METVVRRCPF LSRVPQAFLQ KAGKSLLFYA QNCPKMMEVG AKPAPRTLST    50
SAVHCQQVKE TPPANEKEKT AKAAVQQAPD ESQMAQTPDG TQLPSGHPSP 100
ATSQGSGSKC PFLAAQLSQT GSSVFRKASL ELQEDVQEMH AVRKEAAQSP 150
VPPSLVNVKT DGEDPSRLLK NFQDIMRKQR PERVSHLLQD NLPKSVSTFQ 200
YDHFFEKKID EKKNDHTYRV FKTVNRRAQI FPMADDYTDS LITKKQVSVW 250
CSNDYLGMSR HPRVCGAVME TVKQHGAGAG GTRNISGTSK FHVELEQALA 300
DLHGKDAALL FSSCFVANDS TLFTLAKMMP GCEIYSDSGN HASMIQGIRN 350
SRVPKYIFRH NDVNHLRELL QRSDPSVPKI VAFETVHSMD GAVCPLEELC 400
DVAHEFGAIT FVDEVHAVGL YGARGGGIGD RDGVMPKMDI ISGTLGKAFG 450
CVGGYIASTS LLIDTVRSYA AGFIFTTSLP PMLLAGALES VRILKSSEGR 500
ALRRQHQRNV KLLRQMLMDA GLPVIHCPSH IIPVRVADAA KNTEICDELM 550
TRHNIYVQAI NYPTVPRGEE LLRIAPTPHH TPQMMNFFVE KLLVTWKRVG 600
LELKPHSSAE CNFCRRPLHF EVMSEREKAY FSGMSKMVSA QA 642
Length:642
Mass (Da):71,018
Last modified:September 27, 2004 - v2
Checksum:i34E0CBEB8599CFE5
GO

Sequence conflict

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti192 – 1921L → S in AAA91867. 1 Publication
Sequence conflicti391 – 3911Missing in AAH22110. 1 Publication
Sequence conflicti463 – 4631I → T in AAA91867. 1 Publication
Sequence conflicti501 – 5011A → P in AAA91867. 1 Publication
Sequence conflicti602 – 6021E → V in AAA91867. 1 Publication
Sequence conflicti625 – 6251E → V in AAA91867. 1 Publication

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
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

EnsembliENSMUST00000074082; ENSMUSP00000073725; ENSMUSG00000032786.
ENSMUST00000112524; ENSMUSP00000108143; ENSMUSG00000032786.
ENSMUST00000141118; ENSMUSP00000117014; ENSMUSG00000032786.
GeneIDi11655.
KEGGimmu:11655.
UCSCiuc009rjj.2. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
BC022110 mRNA. Translation: AAH22110.1 .
M63245 mRNA. Translation: AAA91867.1 .
CCDSi CCDS40756.2.
RefSeqi NP_001278764.1. NM_001291835.1.
NP_065584.2. NM_020559.2.
UniGenei Mm.290578.

3D structure databases

ProteinModelPortali Q8VC19.
SMRi Q8VC19. Positions 201-592.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

STRINGi 10090.ENSMUSP00000117014.

PTM databases

PhosphoSitei Q8VC19.

Proteomic databases

MaxQBi Q8VC19.
PaxDbi Q8VC19.
PRIDEi Q8VC19.

Protocols and materials databases

Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENSMUST00000074082 ; ENSMUSP00000073725 ; ENSMUSG00000032786 .
ENSMUST00000112524 ; ENSMUSP00000108143 ; ENSMUSG00000032786 .
ENSMUST00000141118 ; ENSMUSP00000117014 ; ENSMUSG00000032786 .
GeneIDi 11655.
KEGGi mmu:11655.
UCSCi uc009rjj.2. mouse.

Organism-specific databases

CTDi 211.
MGIi MGI:87989. Alas1.

Phylogenomic databases

eggNOGi COG0156.
GeneTreei ENSGT00530000063111.
HOGENOMi HOG000221020.
HOVERGENi HBG005954.
InParanoidi Q8VC19.
KOi K00643.
OMAi PQMMSYF.
OrthoDBi EOG7TQV0F.
PhylomeDBi Q8VC19.
TreeFami TF300724.

Enzyme and pathway databases

UniPathwayi UPA00251 ; UER00375 .
Reactomei REACT_198602. PPARA activates gene expression.
REACT_203298. Heme biosynthesis.
REACT_205251. Transcriptional activation of mitochondrial biogenesis.

Miscellaneous databases

ChiTaRSi ALAS1. mouse.
NextBioi 279265.
PROi Q8VC19.
SOURCEi Search...

Gene expression databases

ArrayExpressi Q8VC19.
Bgeei Q8VC19.
CleanExi MM_ALAS1.
Genevestigatori Q8VC19.

Family and domain databases

Gene3Di 3.40.640.10. 1 hit.
3.90.1150.10. 1 hit.
InterProi IPR010961. 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 ]
Pfami PF00155. Aminotran_1_2. 1 hit.
PF09029. Preseq_ALAS. 2 hits.
[Graphical view ]
SUPFAMi SSF53383. SSF53383. 1 hit.
TIGRFAMsi TIGR01821. 5aminolev_synth. 1 hit.
PROSITEi PS00599. AA_TRANSFER_CLASS_2. 1 hit.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Strain: FVB/N.
    Tissue: Liver.
  2. Young E.G., Dierks P.M.
    Submitted (MAR-1996) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 188-642.
    Strain: DBA/2.
    Tissue: Liver.

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: September 3, 2014
This is version 106 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

External Data

Dasty 3

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