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Unit Code 80288:
Uroporphyrinogen III Synthase (Co-Synthase) (UPG III S), Erythrocytes

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Useful For

Ruling in/out CEP, a disorder caused by decreased

uroporphyrinogen III synthase activity

 

Not useful for ruling out acute intermittent porphyria, a disorder

caused by decreased uroporphyrinogen I synthase (also

known as porphobilinogen deaminase). For AIP, order #88925

"Porphobilinogen (PBG) Deaminase, Erythrocytes".

Special Instructions and Forms

• Porphyrinogens, Porphyrins, and Porphyrias 
• Porphyria (Acute) Testing Algorithm 
• Porphyria (Cutaneous) Testing Algorithm 

Method Name

High Performance Liquid Chromatography (HPLC)
Note: This test measures UPG III synthase to diagnose
congenital erythropoietic porphyria. It does not
measure UPG I synthase, the enzyme deficiency
responsible for acute intermittent porphyria (AIP).
For AIP (and UPG I synthase), order #88925
"Porphobilinogen (PBG) Deaminase, Erythrocytes."
See "Porphyria (Acute) Testing Algorithm" and
"Porphyria (Cutaneous) Testing Algorithm" in
Special Instructions for additional information.

Reporting Name

Uroporphyrinogen III Synthase, RBC

Ordering Mnemonic

UPGC

Aliases

Congenital Hemoporphyria

Congenital Porphyria

Congential Erythropoietic Porphyria (CEP)

Erythropoietic Uroporphyria

Gunthers Disease

UPG III S (Uroporphyrinogen III Synthase)

Key