Porphobilinogen

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Porphobilinogen
2D structure for Porphobilinogen
Chemical Name	3-[5-(aminomethyl)-4-(carboxymethyl)-1H-pyrrol-3-yl]propanoic acid
Chemical Formula	C₁₀H₁₄N₂O₄
CAS Number	487-90-1
Chemical Information	HMDB00245
Biochemical Taxonomy	Amino Acids
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Porphyrin and Chlorophyll Metabolism
Biofluid Location	Blood Urine
Tissue Location	Liver Predominantly Erythrocyte
Normal Biofluid Concentrations	Blood: 0.066 +/- 0.067 uM Blood: 2.0 +/- 0.53 uM Urine: 0.29 +/- 0.14 umol/mmol creatinine Urine: 0.31 +/- 0.13 umol/mmol creatinine Urine: <=0.579 umol/mmol creatinine
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Not Available
Other (Monogenic Disorders)	Porphyria variegata OMIM: 176200
Abnormal Biofluid Concentrations	Not Available
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Introduction

guidelines
Porphobilinogen is a pyrrole involved in porphyrin metabolism. -- Wikipedia; It consists of a pyrrole ring with acetyl, propionyl, and aminomethyl side chains; It is a key monopyrrolic intermediate in porphyrin, chlorophyll and vitamin B12 biosynthesis. Porphobilinogen is generated by the enzyme ALA dehydratase by combining two molecules of dALA together, and converted into hydroxymethyl bilane by the enzyme porphobilinogen deaminase. 4 molecules of porphobilinogen are condensed to form one molecule of uroporphyrinogen III, which is then converted successively to coproporphyrinogen III, protoporphyrin IX, and heme. Porphobilinogen is produced in excess and excreted in the urine in acute intermittent porphyria and several other porphyrias.