PNP

Chr 14

purine nucleoside phosphorylase

Also known as: NP, PRO1837, PUNP

This gene encodes an enzyme which reversibly catalyzes the phosphorolysis of purine nucleosides. The enzyme is trimeric, containing three identical subunits. Mutations which result in nucleoside phosphorylase deficiency result in defective T-cell (cell-mediated) immunity but can also affect B-cell immunity and antibody responses. Neurologic disorders may also be apparent in patients with immune defects. A known polymorphism at aa position 51 that does not affect enzyme activity has been described. A pseudogene has been identified on chromosome 2. [provided by RefSeq, Jul 2008]

GeneReviewsResearchGenerating clinical summary…

Clinical highlights

Gene-disease validity (ClinGen)
purine nucleoside phosphorylase deficiency · ARDefinitivesufficient evidence for diagnostic panels
5
Active trials
613
Pubs (1 yr)
P/LP submissions
P/LP missense
1.15
LOEUF
DN
Mechanism· predicted
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GeneReview available — PNP
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.15LOEUF
pLI 0.000
Z-score 1.14
OE 0.68 (0.421.15)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.44Z-score
OE missense 0.90 (0.791.04)
146 obs / 161.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.68 (0.421.15)
00.351.4
Missense OE?0.90 (0.791.04)
00.61.4
Synonymous OE?1.00
01.21.6
LoF obs/exp: 10 / 14.7Missense obs/exp: 146 / 161.7Syn Z: -0.02

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PNP · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.