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  2. Methylenetetrahydrofolate reductase deficiency - Wikipedia

    en.wikipedia.org/wiki/Methylenetetrahydrofolate...

    MTHFR is the rate-limiting enzyme in the methyl cycle, which includes the conversion of homocysteine into methionine. Defects in variants of MTHFR can therefore lead to hyperhomocysteinemia. [9] There are two common variants of MTHFR deficiency. In the more significant of the two, the individual is homozygous for the 677T polymorphism.

  3. Methylenetetrahydrofolate reductase - Wikipedia

    en.wikipedia.org/wiki/Methylenetetrahydrofolate...

    Methylenetetrahydrofolate reductase (MTHFR) is the rate-limiting enzyme in the methyl cycle, and it is encoded by the MTHFR gene. [5] Methylenetetrahydrofolate reductase catalyzes the conversion of 5,10-methylenetetrahydrofolate to 5-methyltetrahydrofolate, a cosubstrate for homocysteine remethylation to methionine.

  4. Thrombophilia - Wikipedia

    en.wikipedia.org/wiki/Thrombophilia

    Homocysteine levels are determined by mutations in the MTHFR and ... Tests for thrombophilia ... justify the often high cost, [38] unless the testing is ...

  5. Tetrahydrobiopterin deficiency - Wikipedia

    en.wikipedia.org/wiki/Tetrahydrobiopterin_deficiency

    Mutations in the GCH1, PCBD1, PTS and QDPR genes directly cause BH4 deficiency. Additionally, mutations of the MTHFR gene (A1298C variant) and DHFR can interfere with the recycling of BH4 and lead to less severe, but still clinifically significant, deficiencies of BH4.

  6. Compound heterozygosity - Wikipedia

    en.wikipedia.org/wiki/Compound_heterozygosity

    Compound heterozygosity reflects the diversity of the mutation base for many autosomal recessive genetic disorders; mutations in most disease-causing genes have arisen many times. This means that many cases of disease arise in individuals who have two unrelated alleles, who technically are heterozygotes , but both the alleles are defective.

  7. Hereditary haemochromatosis - Wikipedia

    en.wikipedia.org/wiki/Hereditary_haemochromatosis

    The prevalence of mutations in iron-metabolism genes varies in different populations. A study of 3,011 unrelated white Australians found that 14% were heterozygous carriers of an HFE mutation, 0.5% were homozygous for an HFE mutation, and only 0.25% of the study population had clinically relevant iron overload.