Anti-Coproporphyrinogen III Oxidase antibody (bs-8860R)

Anti-Coproporphyrinogen III Oxidase antibody

DATASHEET

Host:Rabbit

Target Protein:Coproporphyrinogen III Oxidase

IR:Immunogen Range:361-454/454

Clonality:Polyclonal

Isotype:IgG

Entrez Gene:1371

Swiss Prot:P36551

Source:KLH conjugated synthetic peptide derived from human CPOX/Coproporphyrinogen Oxidase:361-454/454 

Purification:affinity purified by Protein A

Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

Background:CPOX is a 454 amino acid mitochondrial enzyme that is localized to the inner membrane space of erythrocytes. It participates in the sixth step of heme biosynthesis by catalyzing the formation of protoporphyrinogen IX from copropophyrinogen III. Mutations in the gene encoding CPOX are the cause of coproporphyria, an autosomal dominant disease characterized by skin photosensitivity and neurological disturbances. Symptoms are often experienced as attacks, which include severe abdominal and nerve pain. People affected by coproporphyria overexcrete copropophyrinogen III in feces and urine and the enzymatic activity of CPOX is found to be approximately half that of normal, leading to a decrease in overall heme synthesis. There is no cure for coproporphyria, but preventative treatment to relieve symptoms usually involves dietary changes and avoidance of drugs and alcohol.

Size:100ul

Concentration:1mg/ml

Applications:ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
ICC(1:100-500)
IF(1:100-500)

Cross Reactive Species:Human
Mouse
Rat
Chicken
Dog
Pig
Cow
Rabbit
Sheep
.

For research use only. Not intended for diagnostic or therapeutic use.