UROD Human
Description
Product Specs
MGSSHHHHHH SSGLVPRGSH MEANGLGPQG FPELKNDTFL RAAWGEETDY TPVWCMRQAG RYLPEFRETR AAQDFFSTCR SPEACCELTL QPLRRFPLDA AIIFSDILVV PQALGMEVTM VPGKGPSFPE PLREEQDLER LRDPEVVASE LGYVFQAITL TRQRLAGRVP LIGFAGAPWT LMTYMVEGGG SSTMAQAKRW LYQRPQASHQ LLRILTDALV PYLVGQVVAG AQALQLFESH AGHLGPQLFN KFALPYIRDV AKQVKARLRE AGLAPVPMII FAKDGHFALE ELAQAGYEVV GLDWTVAPKK ARECVGKTVT LQVNLDPCAL YASEEEIGQL VKQMLDDFGP HRYIANLGHG LYPDMDPEHV GAFVDAVHKH SRLLRQN.
Product Science Overview
Uroporphyrinogen decarboxylase (UROD) is a crucial enzyme in the heme biosynthetic pathway. It catalyzes the conversion of uroporphyrinogen to coproporphyrinogen by removing four carboxymethyl side chains . This enzyme is essential for the production of heme, a vital component of hemoglobin, myoglobin, and various cytochromes .
The UROD gene is located on chromosome 1 and encodes a protein that is approximately 40.8 kDa in size . The protein consists of a single domain containing a (beta/alpha)8-barrel structure with a deep active site cleft formed by loops at the C-terminal ends of the barrel strands . This structure is crucial for its catalytic activity.
UROD catalyzes the fifth step in the heme biosynthetic pathway. It sequentially decarboxylates the four acetate side chains of uroporphyrinogen to form coproporphyrinogen . This reaction is essential for the proper synthesis of heme, as only coproporphyrinogen III can ultimately be converted to heme .
Deficiency or mutations in the UROD gene can lead to disorders such as porphyria cutanea tarda (PCT) and hepatoerythropoietic porphyria (HEP) . These conditions are characterized by the accumulation of porphyrins in the skin and liver, leading to photosensitivity, skin lesions, and liver dysfunction.
Recombinant UROD is produced using expression systems such as Escherichia coli . This allows for the study of the enzyme’s structure and function in detail. The crystal structure of recombinant human UROD has been determined at a resolution of 1.60 Å, providing insights into its catalytic mechanism and potential therapeutic targets .
