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  2. Hyperlysinemia - Wikipedia

    en.wikipedia.org/wiki/Hyperlysinemia

    Hyperlysinemia has an autosomal recessive pattern of inheritance. Hyperlysinemia is inherited in an autosomal recessive manner. [2] This means the defective gene responsible for the disorder is located on an autosome, and two copies of the defective gene (one inherited from each parent) are required in order to be born with the disorder.

  3. α-Ketoglutaric acid - Wikipedia

    en.wikipedia.org/wiki/Α-Ketoglutaric_acid

    Outside of the citric acid cycle, α-ketoglutarate is made by a) the enzymes isocitrate dehydrogenase 1 or 2 which remove a carboxy group from isocitrate by oxidative decarboxylation to form α-ketoglutarate; b) glutaminolysis in which the enzyme glutaminase removes the amino group (i.e., −NH 2) from glutamine to form glutamate which is ...

  4. Oxoglutarate dehydrogenase complex - Wikipedia

    en.wikipedia.org/wiki/Oxoglutarate_dehydrogenase...

    α-ketoglutarate + NAD + + CoA → Succinyl CoA + CO 2 + NADH Oxoglutarate dehydrogenase (α-Ketoglutarate dehydrogenase) This reaction proceeds in three steps: decarboxylation of α-ketoglutarate, reduction of NAD + to NADH, and subsequent transfer to CoA, which forms the end product, succinyl CoA. ΔG°' for this reaction is -7.2 kcal mol −1.

  5. OGDH - Wikipedia

    en.wikipedia.org/wiki/OGDH

    This gene encodes a subunit that catalyzes the oxidative decarboxylation of alpha-ketoglutarate to Succinyl-CoA at its active site in the fourth step of the citric acid cycle by acting as a base to facilitate the decarboxylation. The main residues responsible for the catalysis are thought to be His 260, Phe 227, Gln685, His 729, Ser302, and His ...

  6. Branched-chain alpha-keto acid dehydrogenase complex

    en.wikipedia.org/wiki/Branched-chain_alpha-keto...

    This enzyme complex catalyzes the oxidative decarboxylation of branched, short-chain alpha-ketoacids. BCKDC is a member of the mitochondrial α-ketoacid dehydrogenase complex family, which also includes pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase, key enzymes that function in the Krebs cycle.

  7. Isocitrate dehydrogenase - Wikipedia

    en.wikipedia.org/wiki/Isocitrate_dehydrogenase

    Isocitrate dehydrogenase (IDH) (EC 1.1.1.42) and (EC 1.1.1.41) is an enzyme that catalyzes the oxidative decarboxylation of isocitrate, producing alpha-ketoglutarate (α-ketoglutarate) and CO 2. This is a two-step process, which involves oxidation of isocitrate (a secondary alcohol ) to oxalosuccinate (a ketone ), followed by the ...

  8. Glutamate dehydrogenase - Wikipedia

    en.wikipedia.org/wiki/Glutamate_dehydrogenase

    NAD + (or NADP +) is a cofactor for the glutamate dehydrogenase reaction, producing α-ketoglutarate and ammonium as a byproduct. [4] [8]Based on which cofactor is used, glutamate dehydrogenase enzymes are divided into the following three classes: [citation needed]

  9. Alpha-aminoadipic semialdehyde synthase - Wikipedia

    en.wikipedia.org/wiki/Alpha-aminoadipic...

    Lysine Degradation Steps Catalyzed by Alpha-aminoadipic semialdehyde dehydrogenase. First, the N-terminal portion of this enzyme which contains lysine-ketoglutarate reductase (LOR/LKR) activity (EC:1.5.1.8) condenses lysine and 2-oxoglutarate to a molecule called saccharopine (Reaction 1 on the figure to the right).

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