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Butylmalonic acid | CAS Number: 534-59-8

Catalog Number
ACM534598
Product Name
Butylmalonic acid
Structure
CAS Number
534-59-8
Synonyms
1,1-Pentanedicarboxylic acid;2-Butylmalonate;2-Butylmalonic acid;2-n-Butylmalonic acid;alpha-Carboxycaproic acid;Butylmalonate;Malonic acid, butyl-;Propanedioic acid, butyl-
Molecular Weight
160.17
Molecular Formula
C7H12O4
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1Ethylmalonic acid impairs brain mitochondrial succinate and malate transport.

Amaral AU1, Cecatto C, Busanello EN, Ribeiro CA, Melo DR, Leipnitz G, Castilho RF, Wajner M.

Mol Genet Metab. 2012 Jan;105(1):84-90. doi: 10.1016/j.ymgme.2011.10.006. Epub 2011 Oct 19.

Tissue accumulation and high urinary excretion of ethylmalonic acid (EMA) occur in ethylmalonic encephalopathy (EE) and short chain acyl-CoA dehydrogenase deficiency (SCADD). Although these autosomal recessive disorders are clinically characterized by neurological abnormalities, the mechanisms underlying the brain damage are poorly known. Considering that little is known about the neurotoxicity of EMA and that hyperlacticacidemia occurs in EE and SCADD, we evaluated the effects of this metabolite on important parameters of oxidative metabolism in isolated rat brain mitochondria. Read More

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Email:
Tel:1-201-478-8534
1-516-662-5404
Fax: 1-516-927-0118
Address: Suite 212, Waverly Plaza, 755 Waverly Avenue, Holtsville, NY 11742, USA