Recombinant human UROS protein

UROS, also known as uroporphyrinogen III synthase, is an enzyme involved in the fourth step of porphyrin metabolism, involved in the conversion of hydroxymethyl bilane into uroporphyrinogen III. Defects in this protein can cause molecular lesions that lead to the autosomal recessive Gunther disease, also known as congenital erythropoietic porphyria (CEP) . Recombinant human uROS protein, fused to His-tag at N-terminus, was expressed in E. coli and purified by using conventional chromatography.

Product Specifications

Product Name Alternative

Uroporphyrinogen-III synthase, uroporphyrinogen III synthase

Expression System

E.coli

Antigen Species

Human

Tag

His-Tag

Applications

SDS-PAGE

Concentration

1 mg/mL (determined by Bradford assay)

Purity

> 95% by SDS-PAGE

Molecular Weight

30.7 kDa (285aa) confirmed by MALDI-TOF

Additionnal Information

UROS, uroporphyrinogen-III synthase, uroporphyrinogen III synthase, ATGP1066-10 µg, ATGP1066-20 µg, ATGP1066-50 µg, ATGP1066-100 µg, ATGP1066-250 µg, ATGP1066-500 µg, ATGP1066-1 mg, ATGP1066-10, ATGP1066-20, ATGP1066-50, ATGP1066-100, ATGP1066-250, ATGP1066-500, ATGP1066-1

References & Citations

Geronimi F. et al. (2003) J. Mol. Med. 81: 310-320.; ; Omata Y. et al. (2004) J. BioChem. 136: 211-220.

Storage Conditions

Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.

Formulation

Liquid in. 20 mM Tris-HCl buffer (pH 8.0) containing 10% glycerol, 0.1M NaCl

Scientific Category

Enzymes & Proteases

NCBI Accession Number

NP_000366

Uniprot Accession Number

P10746

Species

Human

AA Sequence

MKVLLLKDAK EDDCGQDPYI RELGLYGLEA TLIPVLSFEF LSLPSFSEKL SHPEDYGGLI FTSPRAVEAA ELCLEQNNKT EVWERSLKEK WNAKSVYVVG NATASLVSKI GLDTEGETCG NAEKLAEYIC SRESSALPLL FPCGNLKREI LPKALKDKGI AMESITVYQT VAHPGIQGNL NSYYSQQGVP ASITFFSPSG LTYSLKHIQE LSGDNIDQIK FAAIGPTTAR ALAAQGLPVS CTAESPTPQA LATGIRKALQ PHGCC