HEM4_MOUSE
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 03-AUG-2022, entry version 154.
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, Spleen, and
RC 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; P51163; -.
DR PeptideAtlas; P51163; -.
DR PRIDE; P51163; -.
DR ProteomicsDB; 269695; -.
DR Antibodypedia; 32416; 100 antibodies from 21 providers.
DR DNASU; 22276; -.
DR Ensembl; ENSMUST00000033276; ENSMUSP00000033276; ENSMUSG00000030979.
DR Ensembl; ENSMUST00000106145; ENSMUSP00000101751; ENSMUSG00000030979.
DR Ensembl; ENSMUST00000106146; ENSMUSP00000101752; ENSMUSG00000030979.
DR GeneID; 22276; -.
DR KEGG; mmu:22276; -.
DR UCSC; uc009kdf.2; mouse.
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; VNCAQQY; -.
DR OrthoDB; 1557657at2759; -.
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 72 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 270 other tissues.
DR ExpressionAtlas; P51163; baseline and differential.
DR Genevisible; P51163; MM.
DR GO; GO:0005829; C:cytosol; ISO: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:0006783; P:heme biosynthetic process; ISO: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:0046677; P:response to antibiotic; IEA:Ensembl.
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; PTHR12390; 1.
DR Pfam; PF02602; HEM4; 1.
DR SUPFAM; SSF69618; SSF69618; 1.
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
