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dc.contributor.author
Young, Rebecca  
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Jones, Dylan E.  
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Diacovich, Lautaro  
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Witkowski, Andrzej  
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Ryan, Robert O.  
dc.date.available
2022-12-27T16:17:34Z  
dc.date.issued
2021-01  
dc.identifier.citation
Young, Rebecca; Jones, Dylan E.; Diacovich, Lautaro; Witkowski, Andrzej; Ryan, Robert O.; trans-3-Methylglutaconyl CoA isomerization-dependent protein acylation; Academic Press Inc Elsevier Science; Biochemical and Biophysical Research Communications; 534; 1-2021; 261-265  
dc.identifier.issn
0006-291X  
dc.identifier.uri
http://hdl.handle.net/11336/182570  
dc.description.abstract
3-methylglutaconic (3MGC) aciduria is associated with a growing number of discrete inborn errors of metabolism. Herein, an antibody-based approach to detection/quantitation of 3MGC acid has been pursued. When trans-3MGC acid conjugated keyhole limpet hemocyanin (KLH) was inoculated into rabbits a strong immune response was elicited. Western blot analysis provided evidence that immune serum, but not pre-immune serum, recognized 3MGC-conjugated bovine serum albumin (BSA). In competition ELISAs using isolated immune IgG, the limit of detection for free trans-3MGC acid was compared to that for cis-3MGC acid and four structurally related short-chain dicarboxylic acids. Surprisingly, cis-3MGC acid yielded a much lower limit of detection (∼0.1 mg/ml) than trans-3MGC acid (∼1.0 mg/ml) while all other dicarboxylic acids tested were poor competitors. The data suggest trans-3MGC- isomerized during, or after, conjugation to KLH such that the immunogen was actually comprised of KLH harboring a mixture of cis- and trans-3MGC haptens. To investigate this unexpected isomerization reaction, trans-3MGC CoA was prepared and incubated at 37 °C in the presence of BSA. Evidence was obtained that non-enzymatic isomerization of trans-3MGC CoA to cis-3MGC CoA precedes intramolecular catalysis to form cis-3MGC anhydride plus CoASH. Anhydride-dependent acylation of BSA generated 3MGCylated BSA, as detected by anti-3MGC immunoblot. The results presented provide an explanation for the unanticipated detection of 3MGCylated proteins in a murine model of primary 3MGC aciduria. Furthermore, non-enzymatic hydrolysis of cis-3MGC anhydride represents a potential source of cis-3MGC acid found in urine of subjects with 3MGC aciduria.  
dc.format
application/pdf  
dc.language.iso
eng  
dc.publisher
Academic Press Inc Elsevier Science  
dc.rights
info:eu-repo/semantics/restrictedAccess  
dc.rights.uri
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/  
dc.subject
3-METHYLGLUTACONIC ACIDURIA  
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ANTIBODY  
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ENZYME LINKED IMMUNOSORBANT ASSAY  
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HAPTEN  
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ORGANIC ACIDURIA  
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Bioquímica y Biología Molecular  
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Ciencias Biológicas  
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CIENCIAS NATURALES Y EXACTAS  
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Endocrinología y Metabolismo  
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Medicina Clínica  
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CIENCIAS MÉDICAS Y DE LA SALUD  
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Otras Ciencias de la Salud  
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Ciencias de la Salud  
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CIENCIAS MÉDICAS Y DE LA SALUD  
dc.title
trans-3-Methylglutaconyl CoA isomerization-dependent protein acylation  
dc.type
info:eu-repo/semantics/article  
dc.type
info:ar-repo/semantics/artículo  
dc.type
info:eu-repo/semantics/publishedVersion  
dc.date.updated
2022-09-19T16:03:38Z  
dc.journal.volume
534  
dc.journal.pagination
261-265  
dc.journal.pais
Estados Unidos  
dc.description.fil
Fil: Young, Rebecca. University of Nevada; Estados Unidos  
dc.description.fil
Fil: Jones, Dylan E.. University of Nevada; Estados Unidos  
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Fil: Diacovich, Lautaro. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Rosario. Instituto de Biología Molecular y Celular de Rosario. Universidad Nacional de Rosario. Facultad de Ciencias Bioquímicas y Farmacéuticas. Instituto de Biología Molecular y Celular de Rosario; Argentina  
dc.description.fil
Fil: Witkowski, Andrzej. Children’s Hospital Oakland Research Institute; Estados Unidos  
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Fil: Ryan, Robert O.. University of Nevada; Estados Unidos  
dc.journal.title
Biochemical and Biophysical Research Communications  
dc.relation.alternativeid
info:eu-repo/semantics/altIdentifier/url/https://www.sciencedirect.com/science/article/pii/S0006291X20321446?via%3Dihub  
dc.relation.alternativeid
info:eu-repo/semantics/altIdentifier/doi/http://dx.doi.org/10.1016/j.bbrc.2020.11.100