Propionate coa transferase

Because of improvements in therapy, many patients with MMA reach childbearing age. Wasserstein et al. (1999) reported a successful pregnancy and delivery of a healthy baby to a 20-year-old woman with vitamin B12-unresponsive methylmalonic acidemia complicated by moderate renal insufficiency, chronic pancreatitis, anemia, and optic atrophy. Strict metabolic control was maintained throughout her pregnancy. The patient remained clinically asymptomatic during and after delivery, and her metabolic condition remained stable after discharge except for a slight decline in her renal function.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

In propionic acidemia , a rare inherited genetic disorder, propionate acts as a metabolic toxin in liver cells by accumulating in mitochondria as propionyl-CoA and its derivative, methylcitrate, two tricarboxylic acid cycle inhibitors. Propanoate is metabolized oxidatively by glia , which suggests astrocytic vulnerability in propionic acidemia when intramitochondrial propionyl-CoA may accumulate. Propionic acidemia may alter both neuronal and glial gene expression by affecting histone acetylation. [19] [20] When propionic acid is infused directly into rodents' brains, it produces reversible behavior (., hyperactivity , dystonia , social impairment, perseveration ) and brain changes (., innate neuroinflammation, glutathione depletion) that may be used as a means to model autism in rats. [19]

Other names: HMG-CoA reductase inhibitors, statins What are Statins HMG-CoA reductase inhibitors, also known as statins, selectively inhibit an enzyme called HMG-CoA reductase (3-hydroxy-3-methylglutaryl coenzyme A reductase) that is involved in the synthesis of mevalonate, a precursor of sterols including cholesterol. By inhibiting this enzyme, cholesterol and LDL-cholesterol production is decreased. Statins also increase the number of LDL receptors on liver cells, which enhances the uptake and breakdown of LDL-cholesterol. Most of the effects of statins occur in the liver.

Carbon-13 NMR and radiotracer studies were used to determine the precursor to methylmalonate and to study the metabolism of propionate in the cockroach Periplaneta americana. [3,4,5-13C3]Valine labeled carbons 3, 4, and 26 of 3-methylpentacosane, indicating that valine was metabolized via propionyl-CoA to methylmalonyl-CoA and served as the methyl branch unit precursor. Potassium [2-13C]propionate labeled the odd-numbered carbons of hydrocarbons and potassium [3-13C]propionate labeled the even-numbered carbons of hydrocarbons in this insect. This labeling pattern indicates that propionate is metabolized to acetate, with carbon-2 of propionate becoming the methyl carbon of acetate and carbon-3 of propionate becoming the carboxyl carbon of acetate. In vivo studies in which products were separated by HPLC showed that [2-14C]propionate was readily metabolized to acetate. The radioactivity from sodium [1-14C]propionate was not incorporated into succinate nor into any other tricarboxylic acid cycle intermediate, indicating that propionate was not metabolized via methylmalonate to succinate. Similarly, [1-14C]propionate did not label acetate. An experiment designed to determine the subcellular localization of the enzymes involved in converting propionate to acetate showed that they were located in the mitochondrial fraction. Data from both in vivo and in vitro studies as a function of time indicated that propionate was converted directly to acetate and did not first go through tricarboxylic acid cycle intermediates. These data demonstrate a novel pathway of propionate metabolism in insects.

Propionate coa transferase

propionate coa transferase

Other names: HMG-CoA reductase inhibitors, statins What are Statins HMG-CoA reductase inhibitors, also known as statins, selectively inhibit an enzyme called HMG-CoA reductase (3-hydroxy-3-methylglutaryl coenzyme A reductase) that is involved in the synthesis of mevalonate, a precursor of sterols including cholesterol. By inhibiting this enzyme, cholesterol and LDL-cholesterol production is decreased. Statins also increase the number of LDL receptors on liver cells, which enhances the uptake and breakdown of LDL-cholesterol. Most of the effects of statins occur in the liver.

Media:

propionate coa transferasepropionate coa transferasepropionate coa transferasepropionate coa transferasepropionate coa transferase