A description of an HPLC assay for coproporphyrinogen III oxidase activity in mononuclear cells

Journal of Inherited Metabolic Disease

Volumn 26, Issue 6, 2003, Pages 565-570

  Gross, Ulrich ^a   Gerlach, R ^a   Kuhnel A ^b   Seifert, V ^a   Doss, M O ^b  

^a GOETHE UNIVERSITY   (Germany)

^b UNIVERSITY HOSPITAL MARBURG   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

COPROPORPHYRINOGEN OXIDASE; PROTEIN; PROTOPORPHYRIN;

ARTICLE; CONTROLLED STUDY; COPROPORPHYRIA; ENZYME ACTIVITY; ENZYME ASSAY; ENZYME SPECIFICITY; ENZYME SUBSTRATE; FEMALE; HETEROZYGOTE; HIGH PERFORMANCE LIQUID CHROMATOGRAPHY; HUMAN; HUMAN CELL; LIQUID CHROMATOGRAPHY; MALE; MONONUCLEAR CELL; PORPHYRIA;

ADULT; CHROMATOGRAPHY, HIGH PRESSURE LIQUID; COPROPORPHYRINOGEN OXIDASE; COPROPORPHYRINS; FAMILY; FECES; FEMALE; HETEROZYGOTE; HUMANS; MALE; MONOCYTES; PORPHYRIAS; REDUCING AGENTS; REFERENCE VALUES;

EID: 0142182122     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1025952031660     Document Type: Article

References (12)

1. Bøyum A (1984) Separation of lymphocytes, granulocytes, and monocytes from human blood using iodinated density gradient media. Methods Enzymol 108: 88-102.
2. Bradford MM (1976) A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding. Anal Biochem 72: 248-254.
3. Doss MO, Gross U, Lamoril J, et al (1999) Compound heterozygous hereditary coproporphyria with fluorescing teeth. Ann Clin Biochem 36: 680-682.
4. Gray D, Golub SH (1984) Cryopreservation of lymphoid cells. Methods Enzymol 108: 363-367.
5. Groß U, Puy H, Kühnel A, et al (2002a) Molecular, immunological, enzymatic and biochemical studies of coproporphyrinogen oxidase deficiency in a family with hereditary coproporphyria. Cell Mol Biol 48: 49-55.
6. Groß U, Puy H, Meissauer U, et al (2002b) A molecular, enzymatic and clinical study in a family with hereditary coproporphyria. J Inherit Metab Dis 25: 279-286.
7. Guo R, Lim CK, Peters TJ (1988) Accurate and specific HPLC assay of coproporphyrinogen III oxidase activity in human peripheral leucocytes. Clin Chim Acta 177: 245-252.
8. Kühnel A, Groß U, Doss MO (2000) Hereditary coproporphyria in Germany: clinical-biochemical studies in 53 patients. Clin Biochem 33: 465-473.
9. Layer G, Verfurth K, Mahlitz E, Jahn D (2002) Oxygen-independent coproporphyrinogen-III oxidase HemN from Escherichia coli. J Biol Chem 277: 34136-34142.
10. Martasek P (1998) Hereditary coproporphyria. Semin Liver Dis 18: 25-32.
11. Petersen NE, Käehne M, Christiansen L, Brock A, Hother-Nielsen O, Rasmussen K (2000) DGGE analysis of the coproporphyrinogen oxidase gene: two new mutations in DNA from Danish patients with hereditary coproporphyria. Scand J Clin Lab Invest 60: 617-626.
12. Takeuchi H, Kondo M, Daimon M, et al (2001) Neonatal-onset hereditary coproporphyria with male pseudohermaphrodism. Blood 98: 3871-3873.