Uros - uroporphyrinogen III synthase Gene

Also Known as Uros3; UROIIIS

Species: Mus musculus

Gene Type: protein coding
Gene ID: 22276

Summary

The protein encoded by this gene is the fourth enzyme in the heme biosynthesis pathway. It converts hydroxymethylbilane to uroporphyrinogen III, a cyclic tetrapyrrole. This enzyme is defective in the autosomal recessive disorder congenital erythropoietic porphyria. Alternate promoter usage controls cell type-specific expression, including erythroid cell-specific expression. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Sep 2014]

Uros Products (5)

mRNA Protein Name
NM_001302085.1 NP_001289014.1 uroporphyrinogen-III synthase isoform 1
NM_001302086.1 NP_001289015.1 uroporphyrinogen-III synthase isoform 2
NM_001302087.1 NP_001289016.1 uroporphyrinogen-III synthase isoform 1
NM_001360164.1 NP_001347093.1 uroporphyrinogen-III synthase isoform 1
NM_009479.3 NP_033505.1 uroporphyrinogen-III synthase isoform 1

Gene Ontology

  • Molecular Function
  • Biological Process
  • Cellular Component
Molecular Function GO Annotation Evidence References Source
enables uroporphyrinogen-III synthase activity IDA
IDA: Inferred from direct assay
7607680 MGI
enables uroporphyrinogen-III synthase activity IMP
IMP: Inferred from mutant phenotype
16532394 MGI
Biological Process GO Annotation Evidence References Source
involved in heme A biosynthetic process IMP
IMP: Inferred from mutant phenotype
16532394 MGI
involved in heme B biosynthetic process IMP
IMP: Inferred from mutant phenotype
16532394 MGI
involved in heme O biosynthetic process IMP
IMP: Inferred from mutant phenotype
16532394 MGI
acts upstream of or within porphyrin-containing compound biosynthetic process IDA
IDA: Inferred from direct assay
7607680 MGI
Cellular Component GO Annotation Evidence References Source
is active in cytosol IDA
IDA: Inferred from direct assay
24145442 MGI
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern
Protein Preferred Names Protein Names

uroporphyrinogen-III synthase

  • URO-synthase

  • hydroxymethylbilane hydrolyase (cyclizing)

  • uroporphyrinogen-III cosynthase

  • uroporphyrinogen-III cosynthetase

Related Agents

  • Pre-clinical Phase

Pre-clinical Phase

Bioactive Molecules (4)
Cat. No. Product Name
Effect
Purity
Rare Diseases
HY-RS15510 UROS Human Pre-designed siRNA Set A Pre-designed Set / Unkown
HY-RS19972 Uros Mouse Pre-designed siRNA Set A Pre-designed Set / Unkown
HY-RS25435 Hmbs Rat Pre-designed siRNA Set A Pre-designed Set / Unkown
HY-RS26475 Uros Rat Pre-designed siRNA Set A Pre-designed Set / Unkown

Orthologs Information

Species Symbol Source ID
Homo sapiens Uros NCBI NCBI:7390