Discover Community Create You

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

Molecular basis of dimethylglycine dehydrogenase deficiency associated with pathogenic variant H109R.

Journal of inherited metabolic disease - 1 Dec 2008

McAndrew R P, Vockley J, Kim J-J P

Abstract excerpt

Dimethylglycine dehydrogenase (DMGDH) is a mitochondrial matrix flavoprotein that catalyses the demethylation of dimethylglycine to form sarcosine, accompanied by the reduction of the covalently bound FAD cofactor. Electron-transfer flavoprotein reoxidizes the reduced flavin and transfers reducing equivalents to the main mitochondrial respiratory chain through the enzyme ETF-ubiquinone oxidoreductase. DMGDH plays...

Read the complete abstract on PubMed

Topics

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

Share to Oxygen
Share to Chromatography, Ion Exchange
Share to Electrons

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Share to Flavin-Adenine Dinucleotide

Share to Flavoproteins

Share to Metabolism, Inborn Errors

Share to Models, Chemical

Share to Spectrophotometry

Source

Open in PubMed Open full text

Identifiers

PMID: 18937046
DOI: 10.1007/s10545-008-0999-2
PMCID: PMC2828353

Genes

DMGDH

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence. Graph snapshot: 2026-09-29.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Molecular basis of dimethylglycine dehydrogenase deficiency associated with pathogenic variant H109R.PMID 18937046Read ↗

Cloning of dimethylglycine dehydrogenase and a new human inborn error of metabolism, dimethylglycine dehydrogenase deficiency.Semantic proximity Read ↗

Structure and analysis of the human dimethylglycine dehydrogenase gene.Semantic proximity Read ↗

Oxidation of the FAD cofactor to the 8-formyl-derivative in human electron-transferring flavoprotein.Semantic proximity Read ↗

Impeded electron transfer from a pathogenic FMN domain mutant of methionine synthase reductase and its responsiveness to flavin supplementation.Semantic proximity Read ↗

Clear relationship between ETF/ETFDH genotype and phenotype in patients with multiple acyl-CoA dehydrogenation deficiency.Semantic proximity Read ↗

Cysteamine revisited: repair of arginine to cysteine mutations.Semantic proximity Read ↗

Crystal structure of aminomethyltransferase in complex with dihydrolipoyl-H-protein of the glycine cleavage system: implications for recognition of lipoyl protein substrate, disease-related mutations, and reaction mechanism.Semantic proximity Read ↗

Methylamine dehydrogenase: structure and function of electron transfer complexes.Semantic proximity Read ↗

Disruption of the mthfd1 gene reveals a monofunctional 10-formyltetrahydrofolate synthetase in mammalian mitochondria.Semantic proximity Read ↗

Active site aspartate residues are critical for tryptophan tryptophylquinone biogenesis in methylamine dehydrogenase.Semantic proximity Read ↗

NAD- and NADP-dependent mitochondrially targeted methylenetetrahydrofolate dehydrogenase-cyclohydrolases can rescue mthfd2 null fibroblasts.Semantic proximity Read ↗

Human flavin-containing monooxygenase form 3: cDNA expression of the enzymes containing amino acid substitutions observed in individuals with trimethylaminuria.Semantic proximity Read ↗

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