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propionic acid and Propionic Acidemia

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.

Research Excerpts

ExcerptRelevanceReference
"Propionic acidemia is caused by lack of propionyl-CoA carboxylase activity."7.96Disturbance 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."7.72Prenatal 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."3.96Disturbance 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."3.85Inter-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."3.72Prenatal 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."1.43Modulation 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)."1.35Unusual presentation of propionic acidaemia as isolated cardiomyopathy. ( Addonizio, LJ; Barshop, BA; Chung, WK; Lee, TM, 2009)

Research

Studies (8)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's4 (50.00)29.6817
2010's3 (37.50)24.3611
2020's1 (12.50)2.80

Authors

AuthorsStudies
Roginski, AC1
Wajner, A1
Cecatto, C1
Wajner, SM1
Castilho, RF1
Wajner, M1
Amaral, AU1
Wilson, KA1
Han, Y1
Zhang, M1
Hess, JP1
Chapman, KA1
Cline, GW1
Tochtrop, GP1
Brunengraber, H1
Zhang, GF1
Frye, RE1
Rose, S1
Chacko, J1
Wynne, R1
Bennuri, SC1
Slattery, JC1
Tippett, M1
Delhey, L1
Melnyk, S1
Kahler, SG1
MacFabe, DF1
Witters, P1
Debbold, E1
Crivelly, K1
Vande Kerckhove, K1
Corthouts, K1
Debbold, B1
Andersson, H1
Vannieuwenborg, L1
Geuens, S1
Baumgartner, M1
Kozicz, T1
Settles, L1
Morava, E1
Lee, TM1
Addonizio, LJ1
Barshop, BA1
Chung, WK1
Al-Hassnan, ZN1
Boyadjiev, SA1
Praphanphoj, V1
Hamosh, A1
Braverman, NE1
Thomas, GH1
Geraghty, MT1
Yorifuji, T1
Kawai, M1
Mamada, M1
Kurokawa, K1
Egawa, H1
Shigematsu, Y1
Kohno, Y1
Tanaka, K1
Nakahata, T1
Pérez-Cerdá, C1
Pérez, B1
Merinero, B1
Desviat, LR1
Rodríguez-Pombo, P1
Ugarte, M1

Other Studies

8 other studies available for propionic acid and Propionic Acidemia

ArticleYear
Disturbance of bioenergetics and calcium homeostasis provoked by metabolites accumulating in propionic acidemia in heart mitochondria of developing rats.
    Biochimica et biophysica acta. Molecular basis of disease, 2020, 05-01, Volume: 1866, Issue:5

    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.
    American journal of physiology. Endocrinology and metabolism, 2017, 10-01, Volume: 313, Issue:4

    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.
    Translational psychiatry, 2016, 10-25, Volume: 6, Issue:10

    Topics: Autism Spectrum Disorder; Case-Control Studies; Cell Line; Child; Gastrointestinal Microbiome; Human

2016
Autism in patients with propionic acidemia.
    Molecular genetics and metabolism, 2016, Volume: 119, Issue:4

    Topics: Adolescent; Adult; Animals; Autism Spectrum Disorder; Child; Female; Humans; Infant, Newborn; Leucin

2016
Unusual presentation of propionic acidaemia as isolated cardiomyopathy.
    Journal of inherited metabolic disease, 2009, Volume: 32 Suppl 1

    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.
    Journal of inherited metabolic disease, 2003, Volume: 26, Issue:1

    Topics: Acid-Base Equilibrium; Amino Acid Metabolism, Inborn Errors; Bicarbonates; Biomarkers; Diet, Protein

2003
Living-donor liver transplantation for propionic acidaemia.
    Journal of inherited metabolic disease, 2004, Volume: 27, Issue:2

    Topics: Amino Acid Metabolism, Inborn Errors; Child; Child Development; Child, Preschool; Female; Humans; In

2004
Prenatal diagnosis of propionic acidemia.
    Prenatal diagnosis, 2004, Dec-15, Volume: 24, Issue:12

    Topics: Amniotic Fluid; Cells, Cultured; Chorionic Villi; Chromones; DNA Mutational Analysis; Female; Humans

2004