propionic acid has been researched along with Propionic Acidemia in 8 studies
propionic acid : A short-chain saturated fatty acid comprising ethane attached to the carbon of a carboxy group.
Propionic Acidemia: Autosomal recessive metabolic disorder caused by mutations in PROPIONYL-COA CARBOXYLASE genes that result in dysfunction of branch chain amino acids and of the metabolism of certain fatty acids. Neonatal clinical onset is characterized by severe metabolic acidemia accompanied by hyperammonemia, HYPERGLYCEMIA, lethargy, vomiting, HYPOTONIA; and HEPATOMEGALY. Survivors of the neonatal onset propionic acidemia often show developmental retardation, and intolerance to dietary proteins. Late-onset form of the disease shows mild mental and/or developmental retardation, sometimes without metabolic acidemia.
Excerpt | Relevance | Reference |
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"Propionic acidemia is caused by lack of propionyl-CoA carboxylase activity." | 7.96 | Disturbance of bioenergetics and calcium homeostasis provoked by metabolites accumulating in propionic acidemia in heart mitochondria of developing rats. ( Amaral, AU; Castilho, RF; Cecatto, C; Roginski, AC; Wajner, A; Wajner, M; Wajner, SM, 2020) |
"In this report we summarize our experience in prenatal diagnosis of propionic acidemia (PA) since 1987." | 7.72 | Prenatal diagnosis of propionic acidemia. ( Desviat, LR; Merinero, B; Pérez, B; Pérez-Cerdá, C; Rodríguez-Pombo, P; Ugarte, M, 2004) |
"Propionic acidemia is caused by lack of propionyl-CoA carboxylase activity." | 3.96 | Disturbance of bioenergetics and calcium homeostasis provoked by metabolites accumulating in propionic acidemia in heart mitochondria of developing rats. ( Amaral, AU; Castilho, RF; Cecatto, C; Roginski, AC; Wajner, A; Wajner, M; Wajner, SM, 2020) |
"Propionate, 3-hydroxypropionate (3HP), methylcitrate, related compounds, and ammonium accumulate in body fluids of patients with disorders of propionyl-CoA metabolism, such as propionic acidemia." | 3.85 | Inter-relations between 3-hydroxypropionate and propionate metabolism in rat liver: relevance to disorders of propionyl-CoA metabolism. ( Brunengraber, H; Chapman, KA; Cline, GW; Han, Y; Hess, JP; Tochtrop, GP; Wilson, KA; Zhang, GF; Zhang, M, 2017) |
"In this report we summarize our experience in prenatal diagnosis of propionic acidemia (PA) since 1987." | 3.72 | Prenatal diagnosis of propionic acidemia. ( Desviat, LR; Merinero, B; Pérez, B; Pérez-Cerdá, C; Rodríguez-Pombo, P; Ugarte, M, 2004) |
"Propionic acid (PPA) is a ubiquitous short-chain fatty acid, which is a major fermentation product of the enteric microbiome." | 1.43 | Modulation of mitochondrial function by the microbiome metabolite propionic acid in autism and control cell lines. ( Bennuri, SC; Chacko, J; Delhey, L; Frye, RE; Kahler, SG; MacFabe, DF; Melnyk, S; Rose, S; Slattery, JC; Tippett, M; Wynne, R, 2016) |
"Propionic acidaemia (PA) is an autosomal recessive disease that results from deficiency of propionyl-CoA carboxylase (PCC)." | 1.35 | Unusual presentation of propionic acidaemia as isolated cardiomyopathy. ( Addonizio, LJ; Barshop, BA; Chung, WK; Lee, TM, 2009) |
Timeframe | Studies, this research(%) | All Research% |
---|---|---|
pre-1990 | 0 (0.00) | 18.7374 |
1990's | 0 (0.00) | 18.2507 |
2000's | 4 (50.00) | 29.6817 |
2010's | 3 (37.50) | 24.3611 |
2020's | 1 (12.50) | 2.80 |
Authors | Studies |
---|---|
Roginski, AC | 1 |
Wajner, A | 1 |
Cecatto, C | 1 |
Wajner, SM | 1 |
Castilho, RF | 1 |
Wajner, M | 1 |
Amaral, AU | 1 |
Wilson, KA | 1 |
Han, Y | 1 |
Zhang, M | 1 |
Hess, JP | 1 |
Chapman, KA | 1 |
Cline, GW | 1 |
Tochtrop, GP | 1 |
Brunengraber, H | 1 |
Zhang, GF | 1 |
Frye, RE | 1 |
Rose, S | 1 |
Chacko, J | 1 |
Wynne, R | 1 |
Bennuri, SC | 1 |
Slattery, JC | 1 |
Tippett, M | 1 |
Delhey, L | 1 |
Melnyk, S | 1 |
Kahler, SG | 1 |
MacFabe, DF | 1 |
Witters, P | 1 |
Debbold, E | 1 |
Crivelly, K | 1 |
Vande Kerckhove, K | 1 |
Corthouts, K | 1 |
Debbold, B | 1 |
Andersson, H | 1 |
Vannieuwenborg, L | 1 |
Geuens, S | 1 |
Baumgartner, M | 1 |
Kozicz, T | 1 |
Settles, L | 1 |
Morava, E | 1 |
Lee, TM | 1 |
Addonizio, LJ | 1 |
Barshop, BA | 1 |
Chung, WK | 1 |
Al-Hassnan, ZN | 1 |
Boyadjiev, SA | 1 |
Praphanphoj, V | 1 |
Hamosh, A | 1 |
Braverman, NE | 1 |
Thomas, GH | 1 |
Geraghty, MT | 1 |
Yorifuji, T | 1 |
Kawai, M | 1 |
Mamada, M | 1 |
Kurokawa, K | 1 |
Egawa, H | 1 |
Shigematsu, Y | 1 |
Kohno, Y | 1 |
Tanaka, K | 1 |
Nakahata, T | 1 |
Pérez-Cerdá, C | 1 |
Pérez, B | 1 |
Merinero, B | 1 |
Desviat, LR | 1 |
Rodríguez-Pombo, P | 1 |
Ugarte, M | 1 |
8 other studies available for propionic acid and Propionic Acidemia
Article | Year |
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Disturbance of bioenergetics and calcium homeostasis provoked by metabolites accumulating in propionic acidemia in heart mitochondria of developing rats.
Topics: Animals; Calcium; Cardiomyopathies; Cell Fractionation; Cell Line; Energy Metabolism; Humans; Male; | 2020 |
Inter-relations between 3-hydroxypropionate and propionate metabolism in rat liver: relevance to disorders of propionyl-CoA metabolism.
Topics: Acyl Coenzyme A; Ammonium Compounds; Animals; Carbon Isotopes; Citrates; Citric Acid Cycle; Lactic A | 2017 |
Modulation of mitochondrial function by the microbiome metabolite propionic acid in autism and control cell lines.
Topics: Autism Spectrum Disorder; Case-Control Studies; Cell Line; Child; Gastrointestinal Microbiome; Human | 2016 |
Autism in patients with propionic acidemia.
Topics: Adolescent; Adult; Animals; Autism Spectrum Disorder; Child; Female; Humans; Infant, Newborn; Leucin | 2016 |
Unusual presentation of propionic acidaemia as isolated cardiomyopathy.
Topics: Adolescent; Base Sequence; Cardiomyopathies; Heart Transplantation; Humans; Male; Methylmalonyl-CoA | 2009 |
The relationship of plasma glutamine to ammonium and of glycine to acid-base balance in propionic acidaemia.
Topics: Acid-Base Equilibrium; Amino Acid Metabolism, Inborn Errors; Bicarbonates; Biomarkers; Diet, Protein | 2003 |
Living-donor liver transplantation for propionic acidaemia.
Topics: Amino Acid Metabolism, Inborn Errors; Child; Child Development; Child, Preschool; Female; Humans; In | 2004 |
Prenatal diagnosis of propionic acidemia.
Topics: Amniotic Fluid; Cells, Cultured; Chorionic Villi; Chromones; DNA Mutational Analysis; Female; Humans | 2004 |