Mutation update and review of severe methylenetetrahydrofolate reductase deficiency

DS Froese, M Huemer, T Suormala, P Burda… - Human …, 2016 - Wiley Online Library
ABSTRACT Severe 5, 10‐methylenetetrahydrofolate reductase (MTHFR) deficiency is
caused by mutations in the MTHFR gene and results in hyperhomocysteinemia and varying …

Insights into severe 5, 10‐methylenetetrahydrofolate reductase deficiency: molecular genetic and enzymatic characterization of 76 patients

P Burda, A Schäfer, T Suormala, T Rummel… - Human …, 2015 - Wiley Online Library
Methylenetetrahydrofolate reductase (MTHFR) deficiency is the most common inherited
disorder of folate metabolism and causes severe hyperhomocysteinaemia. To better …

[PDF][PDF] Identification of four novel mutations in severe methylenetetrahydrofolate reductase deficiency.

LAJ Kluijtmans, U Wendel, E Stevens… - European Journal of …, 1998 - researchgate.net
Severe methylenetetrahydrofolate reductase (MTHFR) deficiency is an inborn error of folate
metabolism, and is inherited as an autosomal recessive trait. MTHFR is a key enzyme in …

Annotation Molecular genetics of methylenetetrahydrofolate reductase deficiency

R Rozen - Journal of inherited metabolic disease, 1996 - Wiley Online Library
In severe MTHFR deficiency with neonatal or adolescent onset, 9 rare mutations have been
identified. In mild MTHFR deficiency with thermolabile enzyme, a single common mutation …

Clinical pattern, mutations and in vitro residual activity in 33 patients with severe 5, 10 methylenetetrahydrofolate reductase (MTHFR) deficiency

M Huemer, R Mulder-Bleile, P Burda… - Journal of inherited …, 2016 - Springer
Background Severe methylenetetrahydrofolate reductase (MTHFR) deficiency is a rare
inborn defect disturbing the remethylation of homocysteine to methionine (< 200 reported …

Characterization of mutations in severe methylenetetrahydrofolate reductase deficiency reveals an FAD‐responsive mutation

S Sibani, D Leclerc, IS Weisberg, E O'Ferrall… - Human …, 2003 - Wiley Online Library
Abstract Methylenetetrahydrofolate reductase (MTHFR) synthesizes 5‐
methyltetrahydrofolate, a major methyl donor for homocysteine remethylation to methionine …

Relations between molecular and biological abnormalities in 11 families from siblings affected with methylenetetrahydrofolate reductase deficiency

C Tonetti, JM Saudubray, B Echenne… - European journal of …, 2003 - Springer
Methylenetetrahydrofolate reductase (MTHFR) deficiency is an autosomal recessive
disorder resulting in elevated homocysteine levels in plasma and urine. MTHFR catalyses …

The thermolabile variant 677C→ T can further reduce activity when expressed in cis with severe mutations for human methylenetetrahydrofolate reductase

P Goyette, R Rozen - Human mutation, 2000 - Wiley Online Library
Methylenetetrahydrofolate reductase (MTHFR) catalyses the reduction of 5, 10‐
methylenetetrahydrofolate to 5‐methyltetrahydrofolate, a carbon donor for homocysteine …

MTHFR: addressing genetic counseling dilemmas using evidence-based literature

BL Levin, E Varga - Journal of genetic counseling, 2016 - Springer
Abstract The 5, 10 methylenetetrahydrofolate reductase (MTHFR) enzyme is a catalyst in the
folate metabolism pathway, the byproducts of which are involved in the remethylation of …

Characterization of six novel mutations in the methylenetetrahydrofolate reductase (MTHFR) gene in patients with homocystinuria

S Sibani, B Christensen, E O'Ferrall, I Saadi… - Human …, 2000 - Wiley Online Library
Severe deficiency of methylenetetrahydrofolate reductase (MTHFR) is the most common
inborn error of folate metabolism. Patients are characterized by severe …