ID   HEM4_MOUSE              Reviewed;         265 AA.
AC   P51163;
DT   01-OCT-1996, integrated into UniProtKB/Swiss-Prot.
DT   01-OCT-1996, sequence version 1.
DT   27-MAR-2024, entry version 163.
DE   RecName: Full=Uroporphyrinogen-III synthase;
DE            Short=UROIIIS;
DE            Short=UROS;
DE            EC=4.2.1.75 {ECO:0000305|PubMed:7607680};
DE   AltName: Full=Hydroxymethylbilane hydrolyase [cyclizing];
DE   AltName: Full=Uroporphyrinogen-III cosynthase;
GN   Name=Uros; Synonyms=Uros3;
OS   Mus musculus (Mouse).
OC   Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia;
OC   Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae;
OC   Murinae; Mus; Mus.
OX   NCBI_TaxID=10090;
RN   [1]
RP   NUCLEOTIDE SEQUENCE [MRNA], CATALYTIC ACTIVITY, AND PATHWAY.
RC   STRAIN=C57BL/6 X CBA; TISSUE=Liver;
RX   PubMed=7607680; DOI=10.1016/0888-7543(95)80175-l;
RA   Xu W., Kozak C.A., Desnick R.J.;
RT   "Uroporphyrinogen-III synthase: molecular cloning, nucleotide sequence,
RT   expression of a mouse full-length cDNA, and its localization on mouse
RT   chromosome 7.";
RL   Genomics 26:556-562(1995).
RN   [2]
RP   NUCLEOTIDE SEQUENCE [MRNA].
RX   PubMed=7873885; DOI=10.1007/bf00426082;
RA   Bensidhoum M., Ged C.M., Poirier C., Guenet J.-L., de Verneuil H.;
RT   "The cDNA sequence of mouse uroporphyrinogen III synthase and assignment to
RT   mouse chromosome 7.";
RL   Mamm. Genome 5:728-730(1994).
RN   [3]
RP   NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RC   STRAIN=C57BL/6J; TISSUE=Brain;
RX   PubMed=15489334; DOI=10.1101/gr.2596504;
RG   The MGC Project Team;
RT   "The status, quality, and expansion of the NIH full-length cDNA project:
RT   the Mammalian Gene Collection (MGC).";
RL   Genome Res. 14:2121-2127(2004).
RN   [4]
RP   IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
RC   TISSUE=Brain, Brown adipose tissue, Heart, Kidney, Liver, Lung,
RC   Spleen, and Testis;
RX   PubMed=21183079; DOI=10.1016/j.cell.2010.12.001;
RA   Huttlin E.L., Jedrychowski M.P., Elias J.E., Goswami T., Rad R.,
RA   Beausoleil S.A., Villen J., Haas W., Sowa M.E., Gygi S.P.;
RT   "A tissue-specific atlas of mouse protein phosphorylation and expression.";
RL   Cell 143:1174-1189(2010).
CC   -!- FUNCTION: Catalyzes cyclization of the linear tetrapyrrole,
CC       hydroxymethylbilane, to the macrocyclic uroporphyrinogen III, the
CC       branch point for the various sub-pathways leading to the wide diversity
CC       of porphyrins. Porphyrins act as cofactors for a multitude of enzymes
CC       that perform a variety of processes within the cell such as methionine
CC       synthesis (vitamin B12) or oxygen transport (heme) (By similarity).
CC       {ECO:0000250}.
CC   -!- CATALYTIC ACTIVITY:
CC       Reaction=hydroxymethylbilane = H2O + uroporphyrinogen III;
CC         Xref=Rhea:RHEA:18965, ChEBI:CHEBI:15377, ChEBI:CHEBI:57308,
CC         ChEBI:CHEBI:57845; EC=4.2.1.75;
CC         Evidence={ECO:0000305|PubMed:7607680};
CC   -!- PATHWAY: Porphyrin-containing compound metabolism; protoporphyrin-IX
CC       biosynthesis; coproporphyrinogen-III from 5-aminolevulinate: step 3/4.
CC       {ECO:0000305|PubMed:7607680}.
CC   -!- SUBUNIT: Monomer. {ECO:0000250}.
CC   -!- SIMILARITY: Belongs to the uroporphyrinogen-III synthase family.
CC       {ECO:0000305}.
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DR   EMBL; U18867; AAA74742.1; -; mRNA.
DR   EMBL; U16216; AAA79978.1; -; mRNA.
DR   EMBL; U04439; AAA81898.1; -; mRNA.
DR   EMBL; BC068161; AAH68161.1; -; mRNA.
DR   CCDS; CCDS21934.1; -.
DR   PIR; A56838; A56838.
DR   RefSeq; NP_001289014.1; NM_001302085.1.
DR   RefSeq; NP_001289016.1; NM_001302087.1.
DR   RefSeq; NP_033505.1; NM_009479.3.
DR   RefSeq; XP_006507645.1; XM_006507582.3.
DR   AlphaFoldDB; P51163; -.
DR   SMR; P51163; -.
DR   BioGRID; 204462; 1.
DR   STRING; 10090.ENSMUSP00000033276; -.
DR   iPTMnet; P51163; -.
DR   PhosphoSitePlus; P51163; -.
DR   EPD; P51163; -.
DR   PaxDb; 10090-ENSMUSP00000033276; -.
DR   PeptideAtlas; P51163; -.
DR   ProteomicsDB; 269695; -.
DR   Pumba; P51163; -.
DR   Antibodypedia; 32416; 108 antibodies from 22 providers.
DR   DNASU; 22276; -.
DR   Ensembl; ENSMUST00000033276.11; ENSMUSP00000033276.5; ENSMUSG00000030979.14.
DR   Ensembl; ENSMUST00000106145.10; ENSMUSP00000101751.4; ENSMUSG00000030979.14.
DR   Ensembl; ENSMUST00000106146.8; ENSMUSP00000101752.2; ENSMUSG00000030979.14.
DR   GeneID; 22276; -.
DR   KEGG; mmu:22276; -.
DR   UCSC; uc009kdf.2; mouse.
DR   AGR; MGI:98917; -.
DR   CTD; 7390; -.
DR   MGI; MGI:98917; Uros.
DR   VEuPathDB; HostDB:ENSMUSG00000030979; -.
DR   eggNOG; KOG4132; Eukaryota.
DR   GeneTree; ENSGT00390000009853; -.
DR   HOGENOM; CLU_051874_1_1_1; -.
DR   InParanoid; P51163; -.
DR   OMA; CDSMLRG; -.
DR   OrthoDB; 1472480at2759; -.
DR   PhylomeDB; P51163; -.
DR   TreeFam; TF324092; -.
DR   BRENDA; 4.2.1.75; 3474.
DR   Reactome; R-MMU-189451; Heme biosynthesis.
DR   UniPathway; UPA00251; UER00320.
DR   BioGRID-ORCS; 22276; 18 hits in 77 CRISPR screens.
DR   ChiTaRS; Uros; mouse.
DR   PRO; PR:P51163; -.
DR   Proteomes; UP000000589; Chromosome 7.
DR   RNAct; P51163; Protein.
DR   Bgee; ENSMUSG00000030979; Expressed in fetal liver hematopoietic progenitor cell and 273 other cell types or tissues.
DR   ExpressionAtlas; P51163; baseline and differential.
DR   GO; GO:0005829; C:cytosol; IDA:MGI.
DR   GO; GO:0005739; C:mitochondrion; HDA:MGI.
DR   GO; GO:0005542; F:folic acid binding; ISO:MGI.
DR   GO; GO:0004852; F:uroporphyrinogen-III synthase activity; IDA:MGI.
DR   GO; GO:0071418; P:cellular response to amine stimulus; IEA:Ensembl.
DR   GO; GO:0071243; P:cellular response to arsenic-containing substance; IEA:Ensembl.
DR   GO; GO:0006784; P:heme A biosynthetic process; IMP:MGI.
DR   GO; GO:0006785; P:heme B biosynthetic process; IMP:MGI.
DR   GO; GO:0006783; P:heme biosynthetic process; ISO:MGI.
DR   GO; GO:0048034; P:heme O biosynthetic process; IMP:MGI.
DR   GO; GO:0006779; P:porphyrin-containing compound biosynthetic process; IDA:MGI.
DR   GO; GO:0006782; P:protoporphyrinogen IX biosynthetic process; IEA:UniProtKB-UniPathway.
DR   GO; GO:0070541; P:response to platinum ion; IEA:Ensembl.
DR   GO; GO:0006780; P:uroporphyrinogen III biosynthetic process; ISO:MGI.
DR   CDD; cd06578; HemD; 1.
DR   Gene3D; 3.40.50.10090; -; 2.
DR   InterPro; IPR036108; 4pyrrol_syn_uPrphyn_synt_sf.
DR   InterPro; IPR003754; 4pyrrol_synth_uPrphyn_synth.
DR   InterPro; IPR039793; UROS/Hem4.
DR   PANTHER; PTHR12390; UROPORPHYRINOGEN III SYNTHASE; 1.
DR   PANTHER; PTHR12390:SF0; UROPORPHYRINOGEN-III SYNTHASE; 1.
DR   Pfam; PF02602; HEM4; 1.
DR   SUPFAM; SSF69618; HemD-like; 1.
DR   Genevisible; P51163; MM.
PE   1: Evidence at protein level;
KW   Heme biosynthesis; Lyase; Porphyrin biosynthesis; Reference proteome.
FT   CHAIN           1..265
FT                   /note="Uroporphyrinogen-III synthase"
FT                   /id="PRO_0000135252"
SQ   SEQUENCE   265 AA;  28504 MW;  45BD2A6BD709A126 CRC64;
     MKVLLLKDAK EDDSGLDPYI QELRLCGLEA TLIPVLSFEF MSLPSLSEKL SHPEGFGGLI
     FTSPRAVEAV KLCLEKDNKT EAWEKSLKDR WNAKSVYVVG SATASLVNKI GLDAEGAGSG
     NAEKLAEYIC SKPSSELPLL FPCGTIKGDT LPKMLKDKGI PMESMHVYQT VPHPGIQGSL
     KSYYEDQGIP ASITFFSPSG LKYSLEYIQA LSGSSFDQIK FIAIGPSTTR AMAAKGLPVS
     CTAESPTPQA LAAGIRNVLK PNHCC
//