Abstract

Malic acid is widely distributed in nature comprising up to almost 1% by weight of the content of apples. In mammals it is formed in the penultimate reaction in the Krebs cycle and after conversion to oxaloacetate. Malic acid is absorbed by the gut and has specific plasma membrane transporters. This protocol investigates whether provision of malic acid to patients with defects in the Krebs cycle will lead to clinical improvement. One infant has been treated at this time.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Center for Research Resources (NCRR)
Type
General Clinical Research Centers Program (M01)
Project #
2M01RR000042-36
Application #
5217658
Study Section
Project Start
Project End
Budget Start
Budget End
Support Year
36
Fiscal Year
1996
Total Cost
Indirect Cost

  Publications

Spengler, Erin K; Kleiner, David E; Fontana, Robert J (2017) Vemurafenib-induced granulomatous hepatitis. Hepatology 65:745-748
Heidemann, Lauren; Law, James; Fontana, Robert J (2017) A Text Searching Tool to Identify Patients with Idiosyncratic Drug-Induced Liver Injury. Dig Dis Sci 62:615-625
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Crane, Natania A; Jenkins, Lisanne M; Bhaumik, Runa et al. (2017) Multidimensional prediction of treatment response to antidepressants with cognitive control and functional MRI. Brain 140:472-486
Hertz, Daniel L; Speth, Kelly A; Kidwell, Kelley M et al. (2017) Variable aromatase inhibitor plasma concentrations do not correlate with circulating estrogen concentrations in post-menopausal breast cancer patients. Breast Cancer Res Treat 165:659-668
Hertz, D L; Kidwell, K M; Seewald, N J et al. (2017) Polymorphisms in drug-metabolizing enzymes and steady-state exemestane concentration in postmenopausal patients with breast cancer. Pharmacogenomics J 17:521-527
Kadakia, Kunal C; Kidwell, Kelley M; Seewald, Nicholas J et al. (2017) Prospective assessment of patient-reported outcomes and estradiol and drug concentrations in patients experiencing toxicity from adjuvant aromatase inhibitors. Breast Cancer Res Treat 164:411-419
Law, Ian H; Alam, Osman; Bove, Edward L et al. (2016) Follow-Up of a Prospective Surgical Strategy to Prevent Intra-Atrial Reentrant Tachycardia After the Fontan Operation. Circ Arrhythm Electrophysiol 9:
Schrepf, Andrew; Harper, Daniel E; Harte, Steven E et al. (2016) Endogenous opioidergic dysregulation of pain in fibromyalgia: a PET and fMRI study. Pain 157:2217-2225
As-Sanie, Sawsan; Kim, Jieun; Schmidt-Wilcke, Tobias et al. (2016) Functional Connectivity is Associated With Altered Brain Chemistry in Women With Endometriosis-Associated Chronic Pelvic Pain. J Pain 17:1-13

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