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Disruption of mitochondrial functions and oxidative stress contribute to neurologic dysfunction in organic acidurias.

Abstract：

:Organic acidurias (OADs) are inherited disorders of amino acid metabolism biochemically characterized by accumulation of short-chain carboxylic acids in tissues and biological fluids of the affected patients and clinically by predominant neurological manifestations. Some of these disorders are amenable to treatment, which significantly decreases mortality and morbidity, but it is still ineffective to prevent long-term neurologic and systemic complications. Although pathogenesis of OADs is still poorly established, recent human and animal data, such as lactic acidosis, mitochondrial morphological alterations, decreased activities of respiratory chain complexes and altered parameters of oxidative stress, found in tissues from patients and from genetic mice models with these diseases indicate that disruption of critical mitochondrial functions and oxidative stress play an important role in their pathophysiology. Furthermore, organic acids that accumulate in the most prevalent OADs were shown to compromise bioenergetics, by decreasing ATP synthesis, mitochondrial membrane potential, reducing equivalent content and calcium retention capacity, besides inducing mitochondrial swelling, reactive oxygen and nitrogen species generation and apoptosis. It is therefore presumed that secondary mitochondrial dysfunction and oxidative stress caused by major metabolites accumulating in OADs contribute to tissue damage in these pathologies.

journal_name

Arch Biochem Biophys

journal_title

Archives of biochemistry and biophysics

authors

Wajner M,Vargas CR,Amaral AU

doi

10.1016/j.abb.2020.108646

subject

Has Abstract

pub_date

2020-12-15 00:00:00

pages

108646

eissn

0003-9861

issn

1096-0384

pii

S0003-9861(20)30655-X

journal_volume

696

pub_type

杂志文章,评审

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