Biochemical and molecular characterization of 3-Methylcrotonylglycinuria in an Italian asymptomatic girl

Cozzolino, Claudia; Villani, Guido; Frisso, Giulia; Scolamiero, Emanuela; Albano, Lucia; Gallo, Giovanna; Romanelli, Roberta Maia de Castro; Ruoppolo, Margherita

doi:10.1590/1678-4685-gmb-2017-0093

Cited by 10 publications

(6 citation statements)

References 28 publications

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“… 2 53 Methylcrotonoyl-CoA carboxylase also relates synthesis of fatty acid with Acetyl-CoA carboxylase. 54 Contrary to the previous research indicating that two enzymes in this reaction are inhibited by glucagon 2 53 and interact with Gcg mRNA, we speculate that these enzymes might be involved in inhibition of Gcg mRNA translation or activation of Gcg mRNA decay. Although there are no studies showing that these carboxylases bind RNA directly, they may interact with Gcg mRNA via other RNA-binding proteins.…”

Section: Discussioncontrasting

confidence: 97%

Profiling of RNA-binding Proteins Interacting With Glucagon and Adipokinetic Hormone mRNAs

Yeom

Chun

et al. 2022

J Lipid Atheroscler

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Objective Glucagon in mammals and its homolog (adipokinetic hormone [AKH] in Drosophila melanogaster ) are peptide hormones which regulate lipid metabolism by breaking down triglycerides. Although regulatory mechanisms of glucagon and AKH expression have been widely studied, post-transcriptional gene expression of glucagon has not been investigated thoroughly. In this study, we aimed to profile proteins binding with Gcg messenger RNA (mRNA) in mouse and Akh mRNA in Drosophila. Methods Drosophila Schneider 2 (S2) and mouse 3T3-L1 cell lysates were utilized for affinity pull down of Akh and Gcg mRNA respectively using biotinylated anti-sense DNA oligoes against target mRNAs. Mass spectrometry and computational network analysis revealed mRNA-interacting proteins residing in functional proximity. Results We observed that 1) 91 proteins interact with Akh mRNA from S2 cell lysates, 2) 34 proteins interact with Gcg mRNA from 3T3-L1 cell lysates. 3) Akh mRNA interactome revealed clusters of ribosomes and known RNA-binding proteins (RBPs). 4) Gcg mRNA interactome revealed mRNA-binding proteins including Plekha7, zinc finger protein, carboxylase, lipase, histone proteins and a cytochrome, Cyp2c44. 5) Levels of Gcg mRNA and its interacting proteins are elevated in skeletal muscles isolated from old mice compared to ones from young mice. Conclusion Akh mRNA in S2 cells are under active translation in a complex of RBPs and ribosomes. Gcg mRNA in mouse precursor adipocyte is in a condition distinct from Akh mRNA due to biochemical interactions with a subset of RBPs and histones. We anticipate that our study contributes to investigating regulatory mechanisms of Gcg and Akh mRNA decay, translation, and localization.

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Section: Discussioncontrasting

confidence: 97%

Profiling of RNA-binding Proteins Interacting With Glucagon and Adipokinetic Hormone mRNAs

Yeom

Chun

et al. 2022

J Lipid Atheroscler

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“… 4 MCC deficiency can lead to the disorder of leucine metabolism, which can be manifested in a variety of phenotypes, including neurological disorders, infant death and asymptomatic adults. 5 – 8 A mouse study has shown that MCCC2 is associated with obesity, insulin resistance and dyslipidemia. 9 In addition, MCC deletion indicates changes in glycolysis, TCA cycle, OXPHOS, gluconeogenesis, β-oxidation and branched chain fatty acid metabolism.…”

Section: Introductionmentioning

confidence: 99%

<p>Methylcrotonoyl-CoA Carboxylase 2 Promotes Proliferation, Migration and Invasion and Inhibits Apoptosis of Prostate Cancer Cells Through Regulating GLUD1-P38 MAPK Signaling Pathway</p>

Jian-xin

Mao

Huang

et al. 2020

OTT

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Prostate cancer (PCa) is the most common cancer in American men, and the mechanisms of development and progression are still not completely clear. Methylcrotonoyl-CoA carboxylase 2 (MCCC2) was previously identified overexpressed in PCa with lymph node metastasis, but its specific role and mechanisms need further investigation. This study aimed to investigate the role of MCCC2 in PCa cells and its underlying mechanisms. Materials and Methods: Quantitative RT-PCR and Western blotting were used to detect MCCC2 mRNA and protein expression in normal prostate epithelium and cancerous cells. Upon manipulation of MCCC2 expression, cell proliferation was measured by CCK-8 assays and migration and invasion were determined by transwell assays. Changes of apoptosis, cell cycle and mitochondrial membrane potential were evaluated by flow cytometry. MCCC2-mediated signaling pathways were screened by bioinformatics and verified by RT-PCR and Western blotting. Finally, immunohistochemistry was performed to detect the expression of MCCC2 and glutamate dehydrogenase 1 (GLUD1) in PCa tissues to analyze their correlation. Results: We demonstrated that MCCC2 promoted cell proliferation, migration and invasion but inhibited apoptosis in PCa cells. In addition, MCCC2 in 22Rv1 cells induced mitochondrial damage. In PCa tissues, MCCC2 overexpression associated with lymph node metastasis (P=0.001) and high Gleason scores (P<0.001). MCCC2 positively correlated with GLUD1 expression in PCa tissues (r=0.435, P<0.001). Ectopic overexpression of MCCC2 up-regulated GLUD1 and p38 MAPK expression, whereas inhibition of MCCC2 decreased GLUD1 and p38 MAPK expression. Conclusion: MCCC2 exerts oncogenic function in PCa through regulating GLUD1-p38 MAPK signaling pathway, and it may be a potential treatment target.

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“…PCC, MCC, and PC are all located in the mitochondrial matrix. PCC and MCC enzymes regulate critical steps of amino acid catabolism (and odd-chain FAs for PCC) by respectively producing methylmalonyl-CoA from propionyl-CoA for the catabolism of isoleucine, methionine, valine and threonine [ 106 ] ( Figure 1 ), and 3-methylglutaconyl-CoA from 3-methylcrotonyl-CoA for leucine and isovaleric acid catabolism [ 107 ]. PC also converts pyruvate to oxaloacetate that can be used to replenish the TCA cycle or for the initiation of gluconeogenesis (in liver and kidney) or lipogenesis (adipose tissue, liver as well as brain) [ 107 ].…”

Section: Energy Metabolism and B Vitaminsmentioning

confidence: 99%

Metabolic Therapy of Heart Failure: Is There a Future for B Vitamins?

Piquereau

Boitard

Ventura‐Clapier

et al. 2021

IJMS

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Heart failure (HF) is a plague of the aging population in industrialized countries that continues to cause many deaths despite intensive research into more effective treatments. Although the therapeutic arsenal to face heart failure has been expanding, the relatively short life expectancy of HF patients is pushing towards novel therapeutic strategies. Heart failure is associated with drastic metabolic disorders, including severe myocardial mitochondrial dysfunction and systemic nutrient deprivation secondary to severe cardiac dysfunction. To date, no effective therapy has been developed to restore the cardiac energy metabolism of the failing myocardium, mainly due to the metabolic complexity and intertwining of the involved processes. Recent years have witnessed a growing scientific interest in natural molecules that play a pivotal role in energy metabolism with promising therapeutic effects against heart failure. Among these molecules, B vitamins are a class of water soluble vitamins that are directly involved in energy metabolism and are of particular interest since they are intimately linked to energy metabolism and HF patients are often B vitamin deficient. This review aims at assessing the value of B vitamin supplementation in the treatment of heart failure.

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Biochemical and molecular characterization of 3-Methylcrotonylglycinuria in an Italian asymptomatic girl

Cited by 10 publications

References 28 publications

Profiling of RNA-binding Proteins Interacting With Glucagon and Adipokinetic Hormone mRNAs

Profiling of RNA-binding Proteins Interacting With Glucagon and Adipokinetic Hormone mRNAs

<p>Methylcrotonoyl-CoA Carboxylase 2 Promotes Proliferation, Migration and Invasion and Inhibits Apoptosis of Prostate Cancer Cells Through Regulating GLUD1-P38 MAPK Signaling Pathway</p>

Metabolic Therapy of Heart Failure: Is There a Future for B Vitamins?

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