Coproporfyrinogeen III Oxidase Polyklonale Antilichaam

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.

Coprogen oxidase; COPROPORPHYRIA; Coproporphyrinogen III oxidase; Coproporphyrinogenase; COX; CPO; CPOX; CPX; HEM 6; Hem-6; Hem6; HEM6_HUMAN.

1371

Human, Mouse, Rat

361-454/454

Coproporphyrinogen III Oxidase

Unconjugated

KLH conjugated synthetic peptide derived from human CPOX/Coproporphyrinogen Oxidase

ELISA, IHC-P, IHC-F, IF, ICC/IF

Purified by Protein A.

1µg/µl

ELISA (1:500-1000), IHC-P (1:200-400), IHC-F (1:100-500), IF (), ICC/IF (1:50-100)

0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Unmodified

Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

1371