Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Cysteamine revisited: repair of arginine to cysteine mutations.

Journal of inherited metabolic disease - 1 Jul 2017

Gallego-Villar L, Hannibal Luciana, Häberle J, Thöny B, Ben-Omran T, Nasrallah G K, Dewik Al-N, Kruger W D, Blom H J

Abstract excerpt

Cysteamine is a small aminothiol endogenously derived from coenzyme A degradation. For some decades, synthetic cysteamine has been employed for the treatment of cystinosis, and new uses of the drug continue to emerge. In this review, we discuss the role of cysteamine in cellular and extracellular homeostasis and focus on the potential use of aminothiols to reconstitute the function of proteins harboring arginine...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Apolipoprotein E3
Share to Arginine

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Cystathionine beta-Synthase

Share to Cysteine

Share to Cystinosis

Share to Molecular Conformation

Share to Sulfhydryl Compounds

Share to Thromboplastin

Source

Open in PubMed Open full text

Identifiers

PMID: 28643139
DOI: 10.1007/s10545-017-0060-4
PMCID: PMC5740875

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 1 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Cysteamine revisited: repair of arginine to cysteine mutations.PMID 28643139Read ↗

Cystinosis.Semantic proximity Read ↗

TTI-0102: A Novel Natural Controlled-Release Cysteamine Prodrug for Mitochondrial Disease and CystinosisSemantic proximity Read ↗

Effects of long-term cysteamine treatment in patients with cystinosis.Semantic proximity Read ↗

A sulfenic acid enzyme intermediate is involved in the catalytic mechanism of peptide methionine sulfoxide reductase from Escherichia coli.Semantic proximity Read ↗

TMAO miscompartmentalization is a reversible driver of autism pathophysiologySemantic proximity Read ↗

Mechanism-based inhibition of human persulfide dioxygenase by γ-glutamyl-homocysteinyl-glycine.Semantic proximity Read ↗

How to fix a broken protein: restoring function to mutant human cystathionine β-synthase.Semantic proximity Read ↗

Computation-Guided Rational Design of Cysteine-Less Protein Variants in Engineered hCGL.Semantic proximity Read ↗

Microbial conversion of choline to trimethylamine requires a glycyl radical enzyme.Semantic proximity Read ↗

Lysosomal cystine augments apoptosis and causes the phenotype in cystinosis.Semantic proximity Read ↗

Cysteamine-bicalutamide combination treatment restores alpha-ketoglutarate and corrects proximal tubule phenotype in cystinosisSemantic proximity Read ↗

Intralysosomal cystine accumulation in mice lacking cystinosin, the protein defective in cystinosis.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.