Recombinant fragment, corresponding to amino acids 32-141 of Human DHODH
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Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 - 5 µg/ml. Predicted molecular weight: 43 kDa.
Use a concentration of 3 µg/ml.
Catalyzes the conversion of dihydroorotate to orotate with quinone as electron acceptor.
Pyrimidine metabolism; UMP biosynthesis via de novo pathway; orotate from (S)-dihydroorotate (quinone route): step 1/1.
Involvement in disease
Defects in DHODH are the cause of postaxial acrofacial dysostosis (POADS) [MIM:263750]; also known as Miller syndrome. POADS is characterized by severe micrognathia, cleft lip and/or palate, hypoplasia or aplasia of the posterior elements of the limbs, coloboma of the eyelids and supernumerary nipples. POADS is a very rare disorder: only 2 multiplex families, each consisting of 2 affected siblings born to unaffected, nonconsanguineous parents, have been described among a total of around 30 reported cases.
Belongs to the dihydroorotate dehydrogenase family. Type 2 subfamily.
The uncleaved transit peptide is required for mitochondrial targeting and proper membrane integration.
Khutornenko AA et al. The Role of Dihydroorotate Dehydrogenase in Apoptosis Induction in Response to Inhibition of the Mitochondrial Respiratory Chain Complex III. Acta Naturae6:69-75 (2014).
Read more (PubMed: 24772329) »