Uroporphyrinogen Decarboxylase Recombinant Protein | UROD recombinant protein
Recombinant Human Uroporphyrinogen Decarboxylase
Sterile filtered colorless solution.
Introduction: UROD is the fifth enzyme in the human heme biosynthetic pathway and is in charge for the transfer of uroporphyrinogen to coproporphyrinogen through the deletion of four carboxymethyl side chains. UROD Mutations and deficiency result in 3 autosomal disorders in humans: familial porphyria cutanea tarda (f-PCT), sporadic porphyria cutanea tarda (s-PCT) and hepatoerythropoietic porphyria (HEP).
NCBI and Uniprot Product Information
NCBI Description
This gene encodes an enzyme in the heme biosynthetic pathway. This enzyme is responsible for catalyzing the conversion of uroporphyrinogen to coproporphyrinogen through the removal of four carboxymethyl side chains. Mutations and deficiency in this enzyme are known to cause familial porphyria cutanea tarda and hepatoerythropoetic porphyria.[provided by RefSeq, Aug 2010]
Uniprot Description
UROD: Catalyzes the decarboxylation of four acetate groups of uroporphyrinogen-III to yield coproporphyrinogen-III. Defects in UROD are the cause of familial porphyria cutanea tarda (FPCT); also known as porphyria cutanea tarda type II. FPCT is an autosomal dominant disorder characterized by light-sensitive dermatitis, with onset in later life. It is associated with the excretion of large amounts of uroporphyrin in the urine. Iron overload is often present in association with varying degrees of liver damage. Besides the familial form of PCT, a relatively common idiosyncratic form is known in which only the liver enzyme is reduced. This form is referred to as porphyria cutanea tarda sporadic type or type I. PCT type I occurs sporadically as an unusual accompaniment of common hepatic disorders such as alcohol- associated liver disease. Defects in UROD are the cause of hepatoerythropoietic porphyria (HEP). HEP is a rare autosomal recessive disorder. It is the severe form of cutaneous porphyria, and presents in infancy. The level of UROD is very low in erythrocytes and cultured skin fibroblasts, suggesting that HEP is the homozygous state for porphyria cutanea tarda. Belongs to the uroporphyrinogen decarboxylase family.
Protein type: Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; EC 4.1.1.37; Lyase
Chromosomal Location of Human Ortholog: 1p34
Cellular Component: nucleoplasm; cytoplasm; cytosol
Molecular Function: uroporphyrinogen decarboxylase activity
Biological Process: porphyrin metabolic process; protoporphyrinogen IX biosynthetic process; heme biosynthetic process
Disease: Porphyria Cutanea Tarda
Research Articles on UROD
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Product Notes
The UROD urod (Catalog #AAA144079) is a Recombinant Protein produced from E Coli and is intended for research purposes only. The product is available for immediate purchase. The amino acid sequence is listed below: MGSSHHH HHH 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. It is sometimes possible for the material contained within the vial of "Uroporphyrinogen Decarboxylase, Recombinant Protein" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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