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Protein

Porphobilinogen deaminase

Gene

Hmbs

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.

Miscellaneous

The porphobilinogen subunits are added to the dipyrromethane group.By similarity

Catalytic activityi

4 porphobilinogen + H2O = hydroxymethylbilane + 4 NH3.

Cofactori

dipyrromethaneNote: Binds 1 dipyrromethane group covalently.

Pathwayi: protoporphyrin-IX biosynthesis

This protein is involved in step 2 of the subpathway that synthesizes coproporphyrinogen-III from 5-aminolevulinate.
Proteins known to be involved in the 4 steps of the subpathway in this organism are:
  1. Delta-aminolevulinic acid dehydratase (Alad)
  2. Porphobilinogen deaminase (Hmbs)
  3. Uroporphyrinogen-III synthase (Uros)
  4. Uroporphyrinogen decarboxylase (Urod)
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 coproporphyrinogen-III from 5-aminolevulinate, the pathway protoporphyrin-IX biosynthesis and in Porphyrin-containing compound metabolism.

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionTransferase
Biological processHeme biosynthesis, Porphyrin biosynthesis

Enzyme and pathway databases

BRENDAi2.5.1.61 3474
ReactomeiR-MMU-189451 Heme biosynthesis
UniPathwayi
UPA00251;UER00319

Names & Taxonomyi

Protein namesi
Recommended name:
Porphobilinogen deaminase (EC:2.5.1.61)
Short name:
PBG-D
Alternative name(s):
Hydroxymethylbilane synthase
Short name:
HMBS
Pre-uroporphyrinogen synthase
Gene namesi
Name:Hmbs
Synonyms:Uros1
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 9

Organism-specific databases

MGIiMGI:96112 Hmbs

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

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedBy similarity
ChainiPRO_00001430352 – 361Porphobilinogen deaminaseAdd BLAST360

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylserineBy similarity1
Modified residuei69PhosphoserineBy similarity1
Modified residuei74N6-acetyllysineCombined sources1
Modified residuei147PhosphoserineBy similarity1
Modified residuei261S-(dipyrrolylmethanemethyl)cysteineBy similarity1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

MaxQBiP22907
PaxDbiP22907
PeptideAtlasiP22907
PRIDEiP22907

PTM databases

iPTMnetiP22907
PhosphoSitePlusiP22907

Expressioni

Gene expression databases

BgeeiENSMUSG00000032126 Expressed in 272 organ(s), highest expression level in liver
CleanExiMM_HMBS
ExpressionAtlasiP22907 baseline and differential
GenevisibleiP22907 MM

Interactioni

Protein-protein interaction databases

BioGridi200321, 2 interactors
IntActiP22907, 3 interactors
MINTiP22907
STRINGi10090.ENSMUSP00000076575

Structurei

3D structure databases

ProteinModelPortaliP22907
SMRiP22907
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the HMBS family.Curated

Phylogenomic databases

eggNOGiKOG2892 Eukaryota
COG0181 LUCA
GeneTreeiENSGT00390000009083
HOGENOMiHOG000228587
HOVERGENiHBG000967
InParanoidiP22907
KOiK01749
OrthoDBiEOG091G0B9U
TreeFamiTF105389

Family and domain databases

Gene3Di3.30.160.40, 1 hit
HAMAPiMF_00260 Porphobil_deam, 1 hit
InterProiView protein in InterPro
IPR000860 HemC
IPR022419 Porphobilin_deaminase_cofac_BS
IPR022417 Porphobilin_deaminase_N
IPR022418 Porphobilinogen_deaminase_C
IPR036803 Porphobilinogen_deaminase_C_sf
PANTHERiPTHR11557 PTHR11557, 1 hit
PfamiView protein in Pfam
PF01379 Porphobil_deam, 1 hit
PF03900 Porphobil_deamC, 1 hit
PIRSFiPIRSF001438 4pyrrol_synth_OHMeBilane_synth, 1 hit
PRINTSiPR00151 PORPHBDMNASE
SUPFAMiSSF54782 SSF54782, 1 hit
TIGRFAMsiTIGR00212 hemC, 1 hit
PROSITEiView protein in PROSITE
PS00533 PORPHOBILINOGEN_DEAM, 1 hit

Sequences (2+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 2 described isoforms and 3 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: P22907-1) [UniParc]FASTAAdd to basket
Also known as: Non-erythropoietic

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MSGNGGAATT AEENGSKMRV IRVGTRKSQL ARIQTDTVVA MLKALYPGIQ
60 70 80 90 100
FEIIAMSTTG DKILDTALSK IGEKSLFTKE LENALEKNEV DLVVHSLKDV
110 120 130 140 150
PTILPPGFTI GAICKRENPC DAVVFHPKFI GKTLETLPEK SAVGTSSLRR
160 170 180 190 200
VAQLQRKFPH LEFKSIRGNL NTRLRKLDEL QEFSAIVLAV AGLQRMGWQN
210 220 230 240 250
RVGQILHPEE CMYAVGQGAL AVEVRAKDQD ILDLVSVLHD PETLLRCIAE
260 270 280 290 300
RAFLRHLEGG CSVPVAVHTV MKDGQLYLTG GVWSLDGSDS MQETMQATIQ
310 320 330 340 350
VPVQQEDGPE DDPQLVGITA RNIPRGAQLA AENLGISLAS LLLNKGAKNI
360
LDVARQLNDV R
Length:361
Mass (Da):39,344
Last modified:July 27, 2011 - v2
Checksum:iF184C439F4F7B55D
GO
Isoform 2 (identifier: P22907-2) [UniParc]FASTAAdd to basket
Also known as: Erythrocyte

The sequence of this isoform differs from the canonical sequence as follows:
     1-17: Missing.

Show »
Length:344
Mass (Da):37,781
Checksum:i012E4FE8355D775E
GO

Computationally mapped potential isoform sequencesi

There are 3 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A1L1ST19A0A1L1ST19_MOUSE
Porphobilinogen deaminase
Hmbs
126Annotation score:
A0A1L1SRM0A0A1L1SRM0_MOUSE
Porphobilinogen deaminase
Hmbs
52Annotation score:
A0A1L1SU93A0A1L1SU93_MOUSE
Porphobilinogen deaminase
Hmbs
39Annotation score:

Sequence cautioni

The sequence AAA39890 differs from that shown. Reason: Erroneous gene model prediction.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti36D → E in AAA39890 (PubMed:2768242).Curated1
Sequence conflicti36D → E in AAA39891 (PubMed:2768242).Curated1
Sequence conflicti64L → V in AAA39890 (PubMed:2768242).Curated1
Sequence conflicti64L → V in AAA39891 (PubMed:2768242).Curated1
Sequence conflicti117E → Q in AAA39890 (PubMed:2768242).Curated1
Sequence conflicti117E → Q in AAA39891 (PubMed:2768242).Curated1
Sequence conflicti160H → N in AAA39890 (PubMed:2768242).Curated1
Sequence conflicti160H → N in AAA39891 (PubMed:2768242).Curated1
Sequence conflicti271M → I in AAA39890 (PubMed:2768242).Curated1
Sequence conflicti271M → I in AAA39891 (PubMed:2768242).Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0020681 – 17Missing in isoform 2. CuratedAdd BLAST17

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M28666
, M28663, M28664, M28665 Genomic DNA Translation: AAA39890.1 Sequence problems.
M28666
, M28663, M28664, M28665 Genomic DNA Translation: AAA39891.1
AK166734 mRNA Translation: BAE38979.1
AK167702 mRNA Translation: BAE39746.1
CCDSiCCDS23106.1 [P22907-1]
CCDS52779.1 [P22907-2]
PIRiA36513 IBMSN
RefSeqiNP_038579.2, NM_013551.2 [P22907-1]
UniGeneiMm.247676

Genome annotation databases

EnsembliENSMUST00000077353; ENSMUSP00000076575; ENSMUSG00000032126 [P22907-1]
ENSMUST00000097558; ENSMUSP00000095166; ENSMUSG00000032126 [P22907-2]
GeneIDi15288
KEGGimmu:15288
UCSCiuc009pcz.2 mouse [P22907-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M28666
, M28663, M28664, M28665 Genomic DNA Translation: AAA39890.1 Sequence problems.
M28666
, M28663, M28664, M28665 Genomic DNA Translation: AAA39891.1
AK166734 mRNA Translation: BAE38979.1
AK167702 mRNA Translation: BAE39746.1
CCDSiCCDS23106.1 [P22907-1]
CCDS52779.1 [P22907-2]
PIRiA36513 IBMSN
RefSeqiNP_038579.2, NM_013551.2 [P22907-1]
UniGeneiMm.247676

3D structure databases

ProteinModelPortaliP22907
SMRiP22907
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi200321, 2 interactors
IntActiP22907, 3 interactors
MINTiP22907
STRINGi10090.ENSMUSP00000076575

PTM databases

iPTMnetiP22907
PhosphoSitePlusiP22907

Proteomic databases

MaxQBiP22907
PaxDbiP22907
PeptideAtlasiP22907
PRIDEiP22907

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000077353; ENSMUSP00000076575; ENSMUSG00000032126 [P22907-1]
ENSMUST00000097558; ENSMUSP00000095166; ENSMUSG00000032126 [P22907-2]
GeneIDi15288
KEGGimmu:15288
UCSCiuc009pcz.2 mouse [P22907-1]

Organism-specific databases

CTDi3145
MGIiMGI:96112 Hmbs

Phylogenomic databases

eggNOGiKOG2892 Eukaryota
COG0181 LUCA
GeneTreeiENSGT00390000009083
HOGENOMiHOG000228587
HOVERGENiHBG000967
InParanoidiP22907
KOiK01749
OrthoDBiEOG091G0B9U
TreeFamiTF105389

Enzyme and pathway databases

UniPathwayi
UPA00251;UER00319

BRENDAi2.5.1.61 3474
ReactomeiR-MMU-189451 Heme biosynthesis

Miscellaneous databases

PROiPR:P22907
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000032126 Expressed in 272 organ(s), highest expression level in liver
CleanExiMM_HMBS
ExpressionAtlasiP22907 baseline and differential
GenevisibleiP22907 MM

Family and domain databases

Gene3Di3.30.160.40, 1 hit
HAMAPiMF_00260 Porphobil_deam, 1 hit
InterProiView protein in InterPro
IPR000860 HemC
IPR022419 Porphobilin_deaminase_cofac_BS
IPR022417 Porphobilin_deaminase_N
IPR022418 Porphobilinogen_deaminase_C
IPR036803 Porphobilinogen_deaminase_C_sf
PANTHERiPTHR11557 PTHR11557, 1 hit
PfamiView protein in Pfam
PF01379 Porphobil_deam, 1 hit
PF03900 Porphobil_deamC, 1 hit
PIRSFiPIRSF001438 4pyrrol_synth_OHMeBilane_synth, 1 hit
PRINTSiPR00151 PORPHBDMNASE
SUPFAMiSSF54782 SSF54782, 1 hit
TIGRFAMsiTIGR00212 hemC, 1 hit
PROSITEiView protein in PROSITE
PS00533 PORPHOBILINOGEN_DEAM, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiHEM3_MOUSE
AccessioniPrimary (citable) accession number: P22907
Secondary accession number(s): Q3TIV0
Entry historyiIntegrated into UniProtKB/Swiss-Prot: August 1, 1991
Last sequence update: July 27, 2011
Last modified: November 7, 2018
This is version 157 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

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  3. PATHWAY comments
    Index of metabolic and biosynthesis pathways
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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