Uronic Acid Pathway / Essential Pentosuria : Medical Biochemistry by Dr G Bhanu Prakash

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Uronic Acid Pathway / Essential Pentosuria : Medical Biochemistry

Overview
• Part of carbohydrate metabolism
• Involved in the conversion of sugars and uronic acids
• Primarily functions in the liver

Major Reactions
• Formation of Glucuronic Acid: Uronic acids are converted into glucuronic acid, used in detoxification
• Formation of Ascorbic Acid in Plants: Not applicable in humans
• Formation of L-Gulonic Acid and L-Xylulose: Metabolism of glucose

Importance
• Detoxification: Conjugation with glucuronic acid increases water solubility of compounds
• Excretion of Bilirubin: Glucuronic acid combines with bilirubin for excretion
• Metabolism of Carbohydrates: Converts hexoses into pentoses, useful in various metabolic pathways

Clinical Relevance
• Essential Pentosuria: Linked to a deficiency in the pathway (explained below)
• Drug Metabolism: Many drugs are conjugated with glucuronic acid for excretion

Essential Pentosuria

Overview
• A rare, benign metabolic condition
• Included in Garrod's tetrad (Pentosuria, Albinism, Cystinuria, Alkaptonuria)

Cause
• Deficiency of Xylitol Dehydrogenase: Enzyme involved in the conversion of L-xylulose

Symptoms
• Excretion of L-Xylulose in Urine: Leading to pentose sugars in urine

Diagnosis
• Benedict's Test Positive: Indicates reducing sugar in the urine
• Bial's Test Positive: Specific for pentoses

Alimentary Pentosuria
• Occurs after consumption of large amounts of fruits rich in pentoses, like pears
• Temporary and not linked to enzyme deficiency

Treatment
• Dietary Management: Limiting pentose intake
• Monitoring: Regular check-ups to monitor urinary sugars

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