ArticleThe Journal of clinical investigation1998
A novel form of hereditary myeloperoxidase deficiency linked to endoplasmic reticulum/proteasome degradation.
Article in The Journal of clinical investigation, 1998. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers, 1 of them a synthesis that pooled it.
What it found
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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.
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.
Who cites it
19 citing papers in PubMed, 1 synthesis or guideline pooled it, 86 citations in OpenAlex.
- NQO1, MPO, and the risk of lung cancer: a HuGE review.Genetics in medicine : official journal of the American College of Medical Genetics · 2005Pooled it
- Evaluation of Trace Elements Levels and Construction of Auxiliary Prediction Model in Patients with Diabetes Ketoacidosis in Type 1 Diabetes.Diabetes, metabolic syndrome and obesity : targets and therapy · 2023Article
- Article
- Article
- Chaperoning Endoplasmic Reticulum-Associated Degradation (ERAD) and Protein Conformational Diseases.Cold Spring Harbor perspectives in biology · 2019Review
- Article
- Biosynthesis of human myeloperoxidase.Archives of biochemistry and biophysics · 2018Review
- Calcium homeostasis in diabetes mellitus.Journal of veterinary science · 2017Review
- Structure of human promyeloperoxidase (proMPO) and the role of the propeptide in processing and maturation.The Journal of biological chemistry · 2017Article
- Like iron in the blood of the people: the requirement for heme trafficking in iron metabolism.Frontiers in pharmacology · 2014Review
- Conserved cysteine residues provide a protein-protein interaction surface in dual oxidase (DUOX) proteins.The Journal of biological chemistry · 2013Article
- Proconvertase proteolytic processing of an enzymatically active myeloperoxidase precursor.Archives of biochemistry and biophysics · 2012Article
- Functional consequence of positive selection revealed through rational mutagenesis of human myeloperoxidase.Molecular biology and evolution · 2012Article
- The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiology.Physiological reviews · 2012Review
- Arteriovenous CO2 removal improves survival compared to high frequency percussive and low tidal volume ventilation in a smoke/burn sheep acute respiratory distress syndrome model.Annals of surgery · 2007Article
- Cell biology of membrane trafficking in human disease.International review of cytology · 2006Review
- Arginine to cysteine mutation (R499C) found in a Japanese patient with complete myeloperoxidase deficiency.Gene expression · 2006Article
- Identification of sequences required for the import of human protoporphyrinogen oxidase to mitochondria.The Biochemical journal · 2004Article
- Phospholipase A2 modulates respiratory burst developed by neutrophils in patients with rheumatoid arthritis.Journal of cellular and molecular medicineArticle
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
Myeloperoxidase (MPO) deficiency is a common inherited disorder linked to increased susceptibility to infection and malignancy. We identified a novel missense mutation in the MPO gene at codon 173 whereby tyrosine is replaced with cysteine (Y173C) that is associated with MPO deficiency and assessed its impact on MPO processing and targeting in transfectants expressing normal or mutant proteins. Although the precursor synthesized by cells expressing the Y173C mutation (MPOY173C) was glycosylated, associated with the molecular chaperones calreticulin and calnexin, and acquired heme, it was neither proteolytically processed to mature MPO subunits nor secreted. After prolonged association with calreticulin and calnexin in the endoplasmic reticulum, MPOY173C was degraded. Furthermore, the 20S proteasome inhibitor N-acetyl-L-leucinyl-L-leucinyl-L-norleucinyl inhibited its degradation, suggesting that the proteasome mediates proteolysis of MPOY173C and, thus, participates in quality control in this novel form of hereditary MPO deficiency.
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Registered trials
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.