Metabolism Antibodies 2
Anti-UROD Antibody (CAB5493)
- SKU:
- CAB5493
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Description
Antibody Name: | Anti-UROD Antibody |
Antibody SKU: | CAB5493 |
Antibody Size: | 20uL, 50uL, 100uL |
Application: | WB IF |
Reactivity: | Human, Mouse |
Host Species: | Rabbit |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-367 of human UROD (NP_000365.3). |
Application: | WB IF |
Recommended Dilution: | WB 1:500 - 1:2000 IF 1:50 - 1:200 |
Reactivity: | Human, Mouse |
Positive Samples: | SW620, K-562, SW480, Mouse liver, Mouse kidney, Mouse testis, Mouse heart, HepG2 |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-367 of human UROD (NP_000365.3). |
Purification Method: | Affinity purification |
Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Isotype: | IgG |
Sequence: | MEAN GLGP QGFP ELKN DTFL RAAW GEET DYTP VWCM RQAG RYLP EFRE TRAA QDFF STCR SPEA CCEL TLQP LRRF PLDA AIIF SDIL VVPQ ALGM EVTM VPGK GPSF PEPL REEQ DLER LRDP EVVA SELG YVFQ AITL TRQR LAGR VPLI GFAG APWT LMTY MVEG GGSS TMAQ AKRW LYQR PQAS HQLL RILT DALV PYLV GQVV AGAQ ALQL FESH AGHL GPQL FNKF ALPY IRDV AKQV KARL REAG LAPV PMII FAKD GHFA LEEL AQAG YEVV GLDW TVAP KKAR ECVG KTVT LQGN LDPC ALYA SEEE IGQL VKQM LDDF GPHR YIAN LGHG LYPD MDPE HVGA FVDA VHKH SRLL RQN |
Gene ID: | 7389 |
Uniprot: | P06132 |
Cellular Location: | Cytoplasm |
Calculated MW: | 40kDa |
Observed MW: | 40kDa |
Synonyms: | UROD, PCT, UPD |
Background: | 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. |
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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