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HEM4 uroporphyrinogen-III synthase HEM4 [ Saccharomyces cerevisiae S288C ]

Gene ID: 854452, updated on 4-Jan-2025

Summary

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Official Symbol
HEM4
Official Full Name
uroporphyrinogen-III synthase HEM4
Primary source
SGD:S000005804
Locus tag
YOR278W
See related
AllianceGenome:SGD:S000005804; FungiDB:YOR278W; VEuPathDB:YOR278W
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Saccharomyces cerevisiae S288C (strain: S288C)
Lineage
Eukaryota; Fungi; Dikarya; Ascomycota; Saccharomycotina; Saccharomycetes; Saccharomycetales; Saccharomycetaceae; Saccharomyces
Also known as
SLU2
Summary
Enables uroporphyrinogen-III synthase activity. Involved in heme biosynthetic process. Predicted to be active in cytosol. Used to study erythropoietic protoporphyria. Human ortholog(s) of this gene implicated in cutaneous porphyria. Orthologous to human UROS (uroporphyrinogen III synthase). [provided by Alliance of Genome Resources, Jan 2025]
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Genomic context

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See HEM4 in Genome Data Viewer
Location:
chromosome: XV
Exon count:
1
Sequence:
Chromosome: XV; NC_001147.6 (842817..843644)

Chromosome XV - NC_001147.6Genomic Context describing neighboring genes Neighboring gene ncRNA Neighboring gene ncRNA Neighboring gene Rfm1p Neighboring gene putative serine hydrolase

Genomic regions, transcripts, and products

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Genomic Sequence:
NC_001147.6

Go to nucleotide: Graphics FASTA GenBank

Bibliography

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Related articles in PubMed

  1. Isolation of a nuclear yeast gene involved in the mitochondrial import of cytoplasmically synthesized precursor proteins. Langgut W, et al. Curr Genet, 1986. PMID 2834088
  2. Genetic analysis of a respiration-deficient mutant of Saccharomyces cerevisiae lacking all cytochromes and accumulating coproporphyrin. Gunge N, et al. Genetics, 1967 Oct. PMID 5584564, Free PMC Article
  3. A respiration-deficient mutant of Saccharomyces cerevisiae which accumulates porphyrins and lacks cytochromes. Sugimura T, et al. Biochim Biophys Acta, 1966 Feb 28. PMID 5943431
  4. Identification and characterization of the Arabidopsis gene encoding the tetrapyrrole biosynthesis enzyme uroporphyrinogen III synthase. Tan FC, et al. Biochem J, 2008 Mar 1. PMID 18042043
  5. A rAAV2-producing yeast screening model to identify host proteins enhancing rAAV DNA replication and vector yield. Aponte-Ubillus JJ, et al. Biotechnol Prog, 2019 Jan. PMID 30298993

See all (18) citations in PubMed

GeneRIFs: Gene References Into Functions

Pathways from PubChem

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Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Homology

Gene Ontology Provided by SGD

Function Evidence Code Pubs
enables lyase activity IEA
Inferred from Electronic Annotation
more info
  
enables uroporphyrinogen-III synthase activity IBA
Inferred from Biological aspect of Ancestor
more info
  
enables uroporphyrinogen-III synthase activity IEA
Inferred from Electronic Annotation
more info
  
enables uroporphyrinogen-III synthase activity IMP
Inferred from Mutant Phenotype
more info
 PubMed 
enables uroporphyrinogen-III synthase activity ISS
Inferred from Sequence or Structural Similarity
more info
 PubMed 
Process Evidence Code Pubs
involved_in heme biosynthetic process IEA
Inferred from Electronic Annotation
more info
  
involved_in heme biosynthetic process IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in porphyrin-containing compound biosynthetic process IEA
Inferred from Electronic Annotation
more info
  
involved_in protoporphyrinogen IX biosynthetic process IEA
Inferred from Electronic Annotation
more info
  
involved_in uroporphyrinogen III biosynthetic process IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in uroporphyrinogen III biosynthetic process IEA
Inferred from Electronic Annotation
more info
  
Component Evidence Code Pubs
is_active_in cellular_component ND
No biological Data available
more info
  
is_active_in cytosol IBA
Inferred from Biological aspect of Ancestor
more info
  

General protein information

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Preferred Names
uroporphyrinogen-III synthase HEM4
NP_014921.1
  • Uroporphyrinogen III synthase; catalyzes the conversion of hydroxymethylbilane to uroporphyrinogen III, the fourth step in heme biosynthesis; deficiency in the human homolog can result in the disease congenital erythropoietic porphyria

NCBI Reference Sequences (RefSeq)

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Genome Annotation

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference assembly

Genomic

  1. NC_001147.6 Reference assembly

    Range
    842817..843644
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_001183697.1 → NP_014921.1  TPA: uroporphyrinogen-III synthase HEM4 [Saccharomyces cerevisiae S288C]

    See identical proteins and their annotated locations for NP_014921.1

    Status: REVIEWED

    UniProtKB/Swiss-Prot
    D6W2X7, P06174
    UniProtKB/TrEMBL
    A6ZPB4, B3LJU3, B5VSF8, C7GWB3, C8ZH28, G2WNE5, N1NYU0
    Conserved Domains (1) summary
    pfam02602
    Location:26 → 266
    HEM4; Uroporphyrinogen-III synthase HemD
Nucleotide Protein
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
P06174.2

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