Evidence map›Paper›PMID 35339387›Full record

ArticleMolecular genetics and metabolism2022

Modeling the cognitive effects of diet discontinuation in adults with phenylketonuria (PKU) using pegvaliase therapy in PAH-deficient mice.

Shelley R Winn, Sandra Dudley, Tanja Scherer, Nicole Rimann, Beat Thöny, Sydney Boutros, Destine Krenik, Jacob Raber, Cary O Harding

Open access · hybridAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Molecular genetics and metabolism, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
0.9field-weighted citation impact, top 28% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

4 citing papers in PubMed, 9 citations in OpenAlex.

  1. Article
  2. Article
  3. Experimental Animal Models of Phenylketonuria: Pros and Cons.International journal of molecular sciences · 2025
    Review
  4. Therapeutic liver cell transplantation to treat murine PKU.Journal of inherited metabolic disease · 2024
    Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

9 authors at 3 institutions in 2 countries.

Shelley R WinnDepartment of Medical and Molecular Genetics, Oregon Health & Science University, Mailstop L-103, 3181 Sam Jackson Park Rd., Portland, OR 97239, USA.
Sandra DudleyDepartment of Medical and Molecular Genetics, Oregon Health & Science University, Mailstop L-103, 3181 Sam Jackson Park Rd., Portland, OR 97239, USA.
Tanja SchererDepartment of Pediatrics, University of Zurich, Steinwiessstrasse 75, Zurich CH-8032, Switzerland.
Nicole RimannDepartment of Pediatrics, University of Zurich, Steinwiessstrasse 75, Zurich CH-8032, Switzerland.
Beat ThönyDepartment of Pediatrics, University of Zurich, Steinwiessstrasse 75, Zurich CH-8032, Switzerland.
Sydney BoutrosDepartment of Behavioral Neuroscience, Oregon Health & Science University, 3181 Sam Jackson Park Rd., Portland, OR 97239, USA.
Destine KrenikDepartment of Behavioral Neuroscience, Oregon Health & Science University, 3181 Sam Jackson Park Rd., Portland, OR 97239, USA.
Jacob RaberDepartment of Behavioral Neuroscience, Oregon Health & Science University, 3181 Sam Jackson Park Rd., Portland, OR 97239, USA; Departments of Neurology and Radiation Medicine, Division of Neuroscience, ONPRC, Oregon Health & Science University, 3181 Sam Jackson Park Rd., Portland, OR 97239, USA.
Cary O HardingDepartment of Medical and Molecular Genetics, Oregon Health & Science University, Mailstop L-103, 3181 Sam Jackson Park Rd., Portland, OR 97239, USA. Electronic address: hardingc@ohsu.edu.
Oregon Health & Science University · USUniversity of Zurich · CHOregon National Primate Research Center · US

Funding

Novel therapy for monoamine neurotransmitter deficiency in PKUR01NS080866 · NINDS · OREGON HEALTH & SCIENCE UNIVERSITY · PI HARDING, CARY O. · 2012 to 2020
$3.3M
NINDS NIH HHS R01 NS080866
6 · The paper itself

Abstract

Existing phenylalanine hydroxylase (PAH)-deficient mice strains are useful models of untreated or late-treated human phenylketonuria (PKU), as most contemporary therapies can only be initiated after weaning and the pups have already suffered irreversible consequences of chronic hyperphenylalaninemia (HPA) during early brain development. Therefore, we sought to evaluate whether enzyme substitution therapy with pegvaliase initiated near birth and administered repetitively to C57Bl/6-Pah

Indexed as

Phenylalanine HydroxylasePhenylketonuriasAdultAnimalsCognitionDietDisease Models, AnimalFemaleHumansMiceMice, Inbred C57BLPhenylalaninePhenylalanine Ammonia-LyaseRecombinant ProteinspegvaliasePhenylalaninePhenylalanine Ammonia-LyasePhenylalanine HydroxylaseRecombinant ProteinsBehaviorCognitionDopamineHyperphenylalaninemiaPegvaliasePhenylalanine hydroxylasePhenylketonuriaSerotoninTryptophanTryptophan hydroxylaseTyrosineTyrosine hydroxylase

Identifiers

PMID35339387
PMCPMC9106909
OpenAlexW4221014485

What OpenQuestion holds

Textmetadata
LicenceTDM
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.