Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Basket 0
(max 400 entries)x

Your basket is currently empty.

Select item(s) and click on "Add to basket" to create your own collection here
(400 entries max)

Q8VC19

- HEM1_MOUSE

UniProt

Q8VC19 - HEM1_MOUSE

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
    • BLAST
    • Align
    • Format
    • Add to basket
    • History
      Entry version 107 (01 Oct 2014)
      Sequence version 2 (27 Sep 2004)
      Previous versions | rss
    • Help video
    • Feedback
    • Comment

    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 – 2191SubstrateBy similarity
    Binding sitei336 – 3361SubstrateBy similarity
    Binding sitei355 – 3551SubstrateBy similarity
    Binding sitei388 – 3881Pyridoxal phosphateBy similarity
    Binding sitei416 – 4161Pyridoxal phosphateBy similarity
    Binding sitei444 – 4441Pyridoxal phosphateBy similarity
    Active sitei447 – 4471By similarity
    Binding sitei476 – 4761Pyridoxal phosphateBy similarity
    Binding sitei477 – 4771Pyridoxal phosphateBy similarity
    Binding sitei564 – 5641SubstrateBy 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

    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

    Keywords - Cellular componenti

    Mitochondrion

    PTM / Processingi

    Molecule processing

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

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei447 – 4471N6-(pyridoxal phosphate)lysineBy 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.
    ModBaseiSearch...
    MobiDBiSearch...

    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

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti192 – 1921L → S in AAA91867. 1 PublicationCurated
    Sequence conflicti391 – 3911Missing in AAH22110. (PubMed:15489334)Curated
    Sequence conflicti463 – 4631I → T in AAA91867. 1 PublicationCurated
    Sequence conflicti501 – 5011A → P in AAA91867. 1 PublicationCurated
    Sequence conflicti602 – 6021E → V in AAA91867. 1 PublicationCurated
    Sequence conflicti625 – 6251E → V in AAA91867. 1 PublicationCurated

    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

    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: October 1, 2014
    This is version 107 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