2-Methylsuccinic acid( Methylbutanedioic acid | Pyrotartaric Acid )

Catalog No. M19998 CAS No. 498-21-5

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Product Name

2-Methylsuccinic acid
Note

Research use only, not for human use.
Brief Description

Methylsuccinic acid is a normal metabolite found in human fluids. Increased urinary levels of Methylsuccinic acid (together with ethylmalonic acid) are the main biochemical measurable features in ethylmalonic encephalopathy a rare metabolic disorder with an autosomal recessive mode of inheritance that is clinically characterized by neuromotor delay hyperlactic acidemia recurrent petechiae orthostatic acrocyanosis and chronic diarrhea.
Description

Methylsuccinic acid is a normal metabolite found in human fluids. Increased urinary levels of Methylsuccinic acid (together with ethylmalonic acid) are the main biochemical measurable features in ethylmalonic encephalopathy a rare metabolic disorder with an autosomal recessive mode of inheritance that is clinically characterized by neuromotor delay hyperlactic acidemia recurrent petechiae orthostatic acrocyanosis and chronic diarrhea.
In Vitro

2-Methylsuccinic acid is a normal metabolite in human fluids and the main biochemical measurable features in ethylmalonic encephalopathy. The underlying biochemical defect involves isoleucine catabolism.
In Vivo

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Synonyms

Methylbutanedioic acid | Pyrotartaric Acid
Pathway

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Recptor

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Research Area

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Indication

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1.Grosso S et al. Ethylmalonic encephalopathy: further clinical and neuroradiological characterization. J Neurol. 2002 Oct;249(10):1446-50.

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