Anti-CPOX Antibody (CAB3807)
- SKU:
- CAB3807
- Product type:
- Antibody
- Reactivity:
- Human
- Mouse
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Metabolism
Frequently bought together:
Description
Antibody Name: | Anti-CPOX Antibody |
Antibody SKU: | CAB3807 |
Antibody Size: | 20uL, 50uL, 100uL |
Application: | WB IHC |
Reactivity: | Human, Mouse, Rat |
Host Species: | Rabbit |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 111-454 of human CPOX (NP_000088.3). |
Application: | WB IHC |
Recommended Dilution: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 |
Reactivity: | Human, Mouse, Rat |
Positive Samples: | HeLa, A375, HepG2, A-549, Mouse liver, Rat liver |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 111-454 of human CPOX (NP_000088.3). |
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: | TSLG RPEE EEDE LAHR CSSF MAPP VTDL GELR RRPG DMKT KMEL LILE TQAQ VCQA LAQV DGGA NFSV DRWE RKEG GGGI SCVL QDGC VFEK AGVS ISVV HGNL SEEA AKQM RSRG KVLK TKDG KLPF CAMG VSSV IHPK NPHA PTIH FNYR YFEV EEAD GNKQ WWFG GGCD LTPT YLNQ EDAV HFHR TLKE ACDQ HGPD LYPK FKKW CDDY FFIA HRGE RRGI GGIF FDDL DSPS KEEV FRFV QSCA RAVV PSYI PLVK KHCD DSFT PQEK LWQQ LRRG RYVE FNLL YDRG TKFG LFTP GSRI ESIL MSLP LTAR WEYM HSPS ENSK EAEI LEVL RHPR DWVR |
Gene ID: | 1371 |
Uniprot: | P36551 |
Cellular Location: | Mitochondrion intermembrane space |
Calculated MW: | 30kDa/50kDa |
Observed MW: | 36kDa |
Synonyms: | CPOX, CPO, CPX, HCP |
Background: | The protein encoded by this gene is the sixth enzyme of the heme biosynthetic pathway. The encoded enzyme is soluble and found in the intermembrane space of mitochondria. This enzyme catalyzes the stepwise oxidative decarboxylation of coproporphyrinogen III to protoporphyrinogen IX, a precursor of heme. Defects in this gene are a cause of hereditary coproporphyria (HCP). |
UniProt Protein Function: | CPOX: Key enzyme in heme biosynthesis. Catalyzes the oxidative decarboxylation of propionic acid side chains of rings A and B of coproporphyrinogen III. Defects in CPOX are the cause of hereditary coproporphyria (HCP). HCP is an acute hepatic porphyria and an autosomal dominant disease characterized by neuropsychiatric disturbances and skin photosensitivity. Biochemically, there is an overexcretion of coproporphyrin III in the urine and in the feces. HCP is clinically characterized by attacks of abdominal pain, neurological disturbances, and psychiatric symptoms. The symptoms are generally manifested with rapid onset, and can be precipitated by drugs, alcohol, caloric deprivation, infection, endocrine factors or stress. A severe variant form is harderoporphyria, which is characterized by earlier onset attacks, massive excretion of harderoporphyrin in the feces, and a marked decrease of coproporphyrinogen IX oxidase activity. Belongs to the aerobic coproporphyrinogen-III oxidase family. |
UniProt Protein Details: | Protein type:Oxidoreductase; EC 1.3.3.3; Mitochondrial; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll Chromosomal Location of Human Ortholog: 3q12 Cellular Component: mitochondrion; cytoplasm; mitochondrial inner membrane; mitochondrial intermembrane space Molecular Function:coproporphyrinogen oxidase activity; protein homodimerization activity; structural constituent of eye lens Biological Process: response to arsenic; response to methylmercury; response to lead ion; porphyrin metabolic process; response to insecticide; protoporphyrinogen IX biosynthetic process; response to iron ion; heme biosynthetic process Disease: Coproporphyria, Hereditary |
NCBI Summary: | The protein encoded by this gene is the sixth enzyme of the heme biosynthetic pathway. The encoded enzyme is soluble and found in the intermembrane space of mitochondria. This enzyme catalyzes the stepwise oxidative decarboxylation of coproporphyrinogen III to protoporphyrinogen IX, a precursor of heme. Defects in this gene are a cause of hereditary coproporphyria (HCP).[provided by RefSeq, Oct 2009] |
UniProt Code: | P36551 |
NCBI GenInfo Identifier: | 67476671 |
NCBI Gene ID: | 1371 |
NCBI Accession: | P36551.3 |
UniProt Related Accession: | P36551 |
Molecular Weight: | 36kDa |
NCBI Full Name: | Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial |
NCBI Synonym Full Names: | coproporphyrinogen oxidase |
NCBI Official Symbol: | CPOX |
NCBI Official Synonym Symbols: | COX; CPO; CPX; HCP |
NCBI Protein Information: | oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial |
UniProt Protein Name: | Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial |
Protein Family: | Oxygen-dependent coproporphyrinogen-III oxidase |
UniProt Gene Name: | CPOX |
UniProt Entry Name: | HEM6_HUMAN |