Divergent behavior of hydrogen sulfide pools and of the sulfur metabolite lanthionine, a novel uremic toxin, in dialysis patients

Perna, Alessandra F.; Nunzio, Annarita Di; Amoresano, Angela; Pane, F.; Fontanarosa, Carolina; Pucci, Piero; Vigorito, Carmela; Cirillo, Giovanni; Zacchia, Miriam; Trepiccione, Francesco; Ingrosso, Diego

doi:10.1016/j.biochi.2016.04.018

Cited by 39 publications

(65 citation statements)

References 19 publications

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“…The study conducted on our Italian cohort of BBS patients evidenced that 65% of the tested patients did present an estimated glomerular filtration rate (eGFR) higher that 90 ml/min/1.73m 2 , corroborating the findings of the published English study [10]. General medical care of ESRD patients does not differ from normal population; in the literature, several reports describe patients with all modality of renal replacement therapy [13-15]. …”

Section: Renal Abnormalities In Bbssupporting

confidence: 73%

Impact of Local and Systemic Factors on Kidney Dysfunction in Bardet-Biedl Syndrome

Zacchia

Capolongo

Trepiccione

et al. 2017

Kidney Blood Press Res

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Bardet Biedl syndrome (BBS) is a rare inherited syndromic condition characterized by renal and extra-renal disorders. Renal defect, at either structural or functional level, is one of the cardinal clinical features, and is a major cause of morbidity. However, the pathogenic mechanism underlying its dysfunction remains largely unknown, and to date only symptomatic treatment with no specific therapy is available for these patients. Elucidating aberrant cellular and/or systemic processes that impact kidney function is therefore a prerequisite to develop targeted innovative therapeutic strategies for the BBS patients. Given the proven role of BBS proteins in the function of the primary cilium (PC) and considering the clinical overlapping of BBS with other ciliopathies, BBS is considered the result of disruption of ciliary activities. The present review aims at giving an updated overview of the spectrum of renal abnormalities in BBS patients according to the existing scientific literature, and discusses the possible role of intrinsic PC dysfunction into the pathogenesis of renal defects based on the most recent findings demonstrating a possible role of systemic factors in favoring the progression of renal disease.

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Section: Renal Abnormalities In Bbssupporting

confidence: 73%

Impact of Local and Systemic Factors on Kidney Dysfunction in Bardet-Biedl Syndrome

Zacchia

Capolongo

Trepiccione

et al. 2017

Kidney Blood Press Res

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“…[12][13][14] Rather, it has been hypothesized that renal insufficiency causes the accumulation of uremic toxins that subsequently affect homocysteine metabolism, leading to hyperhomocysteinemia. 13,23 Plasma homocysteine concentrations increase with age. In fact, homocysteine levels approximately double from childhood to old age.…”

Section: Hyperhomocysteinemiamentioning

confidence: 99%

The Link Between Hyperhomocysteinemia and Hypomethylation

Barroso

Handy

Castro

2017

Journal of Inborn Errors of Metabolism and Screening

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Increased levels of homocysteine have been established as a risk factor for cardiovascular disease (CVD) by mechanisms still incompletely defined. S-Adenosylhomocysteine (SAH) is the metabolic precursor of homocysteine that accumulates in the setting of hyperhomocysteinemia and is a negative regulator of most cell methyltransferases. Several observations, summarized in the current review, support the concept that SAH, rather than homocysteine, may be the culprit in the CVD risk that has been associated with hyperhomocysteinemia. This review examines the biosynthesis and catabolism of homocysteine and how these pathways regulate accumulation of SAH. In addition, the epidemiological and experimental links between hyperhomocysteinemia and CVD are discussed, along with the evidence suggesting a role for SAH in the disease. Finally, the effects of SAH on the hypomethylation of DNA, RNA, and protein are examined, with an emphasis on how specific molecular targets may be mediators of homocysteine-associated vascular disease.

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“…Diminished levels of plasma H 2 S are also reported to be significantly lowered in CHD patients and in smokers as compared to normal subjects [157], in essential hypertensive children suffering from a metabolic imbalance of homocysteine and hydrogen sulfide [158], and are decreased in patients on chronic haemodialysis due to reduced CSE expression [159]. Lower H 2 S levels also correlate with the accumulation of lanthionine in the blood of uremic patients [160]. These changes potentially contribute to hyperhomocysteinemia in uraemia.…”

Section: Genetic Evidence For a Role Of Cbs Cse And 3-mst In Healthmentioning

confidence: 99%

H2S biosynthesis and catabolism: new insights from molecular studies

Rose

Moore

Zhu

2016

Cell. Mol. Life Sci.

139

100

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Hydrogen sulfide (H2S) has profound biological effects within living organisms and is now increasingly being considered alongside other gaseous signalling molecules, such as nitric oxide (NO) and carbon monoxide (CO). Conventional use of pharmacological and molecular approaches has spawned a rapidly growing research field that has identified H2S as playing a functional role in cell-signalling and post-translational modifications. Recently, a number of laboratories have reported the use of siRNA methodologies and genetic mouse models to mimic the loss of function of genes involved in the biosynthesis and degradation of H2S within tissues. Studies utilising these systems are revealing new insights into the biology of H2S within the cardiovascular system, inflammatory disease, and in cell signalling. In light of this work, the current review will describe recent advances in H2S research made possible by the use of molecular approaches and genetic mouse models with perturbed capacities to generate or detoxify physiological levels of H2S gas within tissues.

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Divergent behavior of hydrogen sulfide pools and of the sulfur metabolite lanthionine, a novel uremic toxin, in dialysis patients

Cited by 39 publications

References 19 publications

Impact of Local and Systemic Factors on Kidney Dysfunction in Bardet-Biedl Syndrome

Impact of Local and Systemic Factors on Kidney Dysfunction in Bardet-Biedl Syndrome

The Link Between Hyperhomocysteinemia and Hypomethylation

H2S biosynthesis and catabolism: new insights from molecular studies

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