Human UROD Recombinant Protein (RPPB2414)

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SKU:
RPPB2414
Product type:
Recombinant Protein
Size:
25ug
Species:
Human
Target:
UROD
Synonyms:
UPD
Synonyms:
PCT
Synonyms:
EC 41137
Synonyms:
URO-D
Source:
Escherichia Coli
Uniprot:
P06132
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Description

Product Name:Human UROD Recombinant Protein
Product Code:RPPB2414
Size:25µg
Species:Human
Target:UROD
Synonyms:UPD, PCT, EC 4.1.1.37, URO-D, UROD, Uroporphyrinogen Decarboxylase.
Source:Escherichia Coli
Physical Appearance:Sterile filtered colorless solution.
Formulation:UROD Human solution containing 20mM Tris pH-8, 1mM DTT, 0.1M NaCl, 1mM EDTA & 20% glycerol.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 95.0% as determined by SDS-PAGE.
Amino Acid Sequence: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

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).

UROD Recombinant Human produced in E.Coli is a single, non-glycosylated polypeptide chain containing 387 amino acids (1-367 a.a.) and having a molecular mass of 43 kDa. The UROD is fused to 20 amino acid His-Tag at N-terminus and purified by proprietary chromatographic techniques.

UniProt Protein Function: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.
UniProt Protein Details:

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

NCBI Summary: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 Code:P06132
NCBI GenInfo Identifier:2507533
NCBI Gene ID:7389
NCBI Accession:P06132.2
UniProt Secondary Accession:P06132,Q16863, Q16883, Q53YB8, Q53ZP6, Q6IB28, Q9BUZ0 A8K762,
UniProt Related Accession:P06132
Molecular Weight:367
NCBI Full Name:Uroporphyrinogen decarboxylase
NCBI Synonym Full Names:uroporphyrinogen decarboxylase
NCBI Official Symbol:UROD  
NCBI Official Synonym Symbols:PCT; UPD  
NCBI Protein Information:uroporphyrinogen decarboxylase; uroporphyrinogen III decarboxylase
UniProt Protein Name:Uroporphyrinogen decarboxylase
UniProt Gene Name:UROD  
UniProt Entry Name:DCUP_HUMAN
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