LP200448-1

7-Isobiopterin

Active

Description

7-Isobiopterin (also known as 7-biopterin or primapterin) [PubChem: 5488923], is a pterin derivative of 4a-hydroxy-BH4. In healthy patients, tetrahydrobiopterin (BH4) is converted to 4a-hydroxy-BH4 with a concomitant conversion of phenylalanine to tyrosine, and BH4 is subsequently regenerated via a pathway that includes pterin-4a-carbinolamine dehydratase (PCD), which is encoded by the PCBD gene. Mutations in the PCBD gene are associated with decreased PCD activity, which results in decreased BH4 salvage, increase in phenylalanine due to inhibition of the BH4 to 4a-hydroxy-BH4 reaction, and concomitant increase in 7-biopterin in urine due to alternate metabolism of 4a-hydroxy-BH4. PMCID: PMC1377166 [UniProt: P61457] Source: Regenstrief LOINC

Basic Part Properties

Part Name

7-Isobiopterin

Part Display Name

7-Isobiopterin

Part Type

Component (Describes the core component or analyte measured)

Created On

2015-09-24

Construct for LOINC Short Name

7-Isobiopterin

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