Succinic acid

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Succinic acid
2D structure for Succinic acid
Chemical Name	Butanedioic acid
Chemical Formula	C₄H₆O₄
CAS Number	110-15-6
Chemical Information	HMDB00254
Biochemical Taxonomy	Dicarboxylic Acids
Functional Taxonomy	DNA Component
Nutritional Taxonomy	Not Available
Metabolic Pathways	Butanoate Metabolism Lysine Degradation Oxidative Phosphorylation Propanoate Metabolism Reductive Carboxylate Cycle (CO2 Fixation) Synthesis and Degradation of Ketone Bodies Valine, Leucine and Isoleucine Degradation
Biofluid Location	Blood Cerebrospinal Fluid (CSF) Saliva Urine
Tissue Location	Brain Fibroblasts Kidney Liver Muscle Pancreas Placenta Prostate Skeletal Muscle Spleen Adipose Tissue
Normal Biofluid Concentrations	Blood: 128.0 (116.0-140.0) uM Blood: 16.0 (0.00-32.0) uM Blood: 8.8 +/- 2.7 uM Cerebrospinal Fluid (CSF): 150.0 +/- 110.0 uM Cerebrospinal Fluid (CSF): 19 uM Cerebrospinal Fluid (CSF): 28.5 (24-33) uM Cerebrospinal Fluid (CSF): 3 +/- 2 uM Cerebrospinal Fluid (CSF): 3.0 +/- 2.0 uM Cerebrospinal Fluid (CSF): 47.2 uM Saliva: 2260 (60.0-4460) uM Urine: 10.9 umol/mmol creatinine Urine: 11.6 umol/mmol creatinine Urine: 185.4 umol/mmol creatinine Urine: 197.2 umol/mmol creatinine Urine: 7.7 umol/mmol creatinine Urine: 7.7 umol/mmol creatinine Urine: 8.250 (0.5-16.0) umol/mmol creatinine
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Canavan Disease Dementia of Alzheimer type (DAT) Lung Cancer
Other (Monogenic Disorders)	Myopathy with deficiency of succinate dehydrogenase and aconitase OMIM: 255125
Abnormal Biofluid Concentrations	Cerebrospinal Fluid (CSF) (Canavan Disease): 19.0 (0.0-38.0) uM Cerebrospinal Fluid (CSF) (Dementia of Alzheimer type (DAT)): 55.0 (19.0-91.0) uM Urine (Lung Cancer): 9.0 +/- 6.0 umol/mmol creatinine
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Introduction

guidelines
Succinic acid is a dicarboxylic acid. The anion, succinate, is a component of the citric acid cycle capable of donating electrons to the electron transfer chain. Succinate dehydrogenase (SDH) plays an important role in the mitochondria, being both part of the respiratory chain and the Krebs cycle. SDH with a covalently attached FAD prosthetic group, binds enzyme substrates (succinate and fumarate) and physiological regulators (oxaloacetate and ATP). Oxidizing succinate links SDH to the fast-cycling Krebs cycle portion where it participates in the breakdown of acetyl-CoA throughout the whole Krebs cycle. The succinate can readily be imported into the mitochondrial matrix by the n-butylmalonate- (or phenylsuccinate-) sensitive dicarboxylate carrier in exchange with inorganic phosphate or another organic acid, e. g. malate. (PMID 16143825) Mutations in the four genes encoding the subunits of the mitochondrial respiratory chain succinate dehydrogenase are associated with a wide spectrum of clinical presentations (i.e.: Huntington's disease. (PMID 11803021)