Catalytic effect of riboflavin on electron transfer from NADH to aquacobalamin

Dereven’kov, Ilia А.; Hannibal, Luciana; Макаров, С. В.; Molodtsov, Pavel A.

doi:10.1007/s00775-019-01745-3

Cited by 5 publications

(3 citation statements)

References 48 publications

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“…As evidenced from in vitro studies, the β-deligated cobalamins undergo transformation into aquacobalamin and remain bound to MMACHC. MMACHC-bound aquacobalamin undergoes reduction to cob(II)alamin in the presence of NADH and riboflavin ( Dereven’kov et al., 2020 ). The reduction involves formation of a NADH⋅Co(III) complex which further decomposes to cob(II)alamin and NADH + .…”

Section: Biochemical Characterization Of the Mmachc Proteinmentioning

confidence: 99%

Versatile enzymology and heterogeneous phenotypes in cobalamin complementation type C disease

et al. 2022

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Section: Biochemical Characterization Of the Mmachc Proteinmentioning

confidence: 99%

Versatile enzymology and heterogeneous phenotypes in cobalamin complementation type C disease

et al. 2022

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“…Methylmalonic acidemia is an autosomal recessive condition 1 characterized by the abnormal accumulation of methylmalonic acid (MMA), which results in cellular and tissue damage, cell apoptosis, and cognitive deficits. 2−4 This condition is due to various genetic defects, including total or partial deficiency of L-methylmalonyl-CoA mutase activity (mut0 and mut-subtypes), defects in the synthesis of the L-methylmalonyl-CoA mutase cofactor (5-adenosylcobalamin; cblA, cblB, cblC, cblD, cblF, and cblG subtypes), and methylmalonyl-CoA epimerase deficiency.…”

Section: ■ Introductionmentioning

confidence: 99%

“…Methylmalonic acidemia is an autosomal recessive condition characterized by the abnormal accumulation of methylmalonic acid (MMA), which results in cellular and tissue damage, cell apoptosis, and cognitive deficits. − This condition is due to various genetic defects, including total or partial deficiency of l -methylmalonyl-CoA mutase activity (mut0 and mut-subtypes), defects in the synthesis of the l -methylmalonyl-CoA mutase cofactor (5-adenosylcobalamin; cblA, cblB, cblC, cblD, cblF, and cblG subtypes), and methylmalonyl-CoA epimerase deficiency . Researchers have shown that during acute metabolic crises, MMA concentrations can increase to approximately 2.5–5.0 mmol/L in plasma and cerebrospinal fluid, leading to severe neurological symptoms such as lethargy, coma, mental delay, and progressive neurological deterioration. − …”

Section: Introductionmentioning

confidence: 99%

Healthy Plasma Exosomes Exert Potential Neuroprotective Effects against Methylmalonic Acid-Induced Hippocampal Neuron Injury

Zhou,

Li,

Song

et al. 2024

ACS Chem. Neurosci.

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Exosomes have shown good potential for alleviating neurological deficits and delaying memory deterioration, but the neuroprotective effects of exosomes remain unknown. Methylmalonic acidemia is a metabolic disorder characterized by the accumulation of methylmalonic acid (MMA) in various tissues that inhibits neuronal survival and function, leading to accelerated neurological deterioration. Effective therapies to mitigate these symptoms are lacking. The purpose of this study was to explore the neuroprotective effects of plasma exosomes on cells and a mouse model of MMA-induced injury. We evaluated the ability of plasma exosomes to reduce the neuronal apoptosis, cross the blood–brain barrier, and affect various parameters related to neuronal function. MMA promoted cell apoptosis, disrupted the metabolic balance, and altered the expression of B-cell lymphoma-2 (Bcl-2), Bcl2-associated X (Bax), and synaptophysin-1 (Syp-1), and these changes may be involved in MMA-induced neuronal apoptosis. Additionally, plasma exosomes normalized learning and memory and protected against MMA-induced neuronal apoptosis. Our findings indicate that neurological deficits are linked to the pathogenesis of methylmalonic acidemia, and healthy plasma exosomes may exert neuroprotective and therapeutic effects by altering the expression of exosomal microRNAs, facilitating neuronal functional recovery in the context of this inherited metabolic disease. Intravenous plasma-derived exosome treatment may be a novel clinical therapeutic strategy for methylmalonic acidemia.

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