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MGCA1 can be distinguished from the other forms of MGCA because the levels of 3-methylglutaconic acid are highly elevated, whereas levels of methylglutaric acid are usually only slightly elevated, and there is a high level of 3-hydroxyisovaleric acid excretion which is not seen in the other forms of MGCA

One key way is through its impact on metabolism

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Borbe JBC, Elvambuena BF, Eugenio FAD, Tan RJM

Afterwards, Cys 63 nucleophilically attacks this sulfide releasing a GS - anion, thereby creating the second GSH