valine and Propionic Acidemia

valine has been researched along with Propionic Acidemia in 4 studies

Research

Studies (4)

Timeframe	Studies, this research(%)	All Research%
pre-1990	0 (0.00)	18.7374
1990's	0 (0.00)	18.2507
2000's	0 (0.00)	29.6817
2010's	3 (75.00)	24.3611
2020's	1 (25.00)	2.80

Authors

Authors	Studies
Chen, H; Cross, JR; DeVita, RJ; Dodatko, T; Dwyer, W; Houten, SM; Leandro, J; Stauffer, B; Vaz, FM; Violante, S; Yu, C	1
Manoli, I; Myles, JG; Venditti, CP	1
Armstrong, AJ; Chapman, KA; Collado, MS; Cui, W; Dash, A; Figler, RA; Hoang, SA; Purdy, M; Simmers, MB; Summar, ML; Wamhoff, BR; Yazigi, NA	1
Karall, D; Sass, JO; Scholl-Bürgi, S; Zschocke, J	1

Reviews

2 review(s) available for valine and Propionic Acidemia

Article	Year
Effects of medical food leucine content in the management of methylmalonic and propionic acidemias. Current opinion in clinical nutrition and metabolic care, 2018, Volume: 21, Issue:1 Topics: Amino Acid Metabolism, Inborn Errors; Animals; Deficiency Diseases; Diet, Protein-Restricted; Foods, Specialized; Homocystinuria; Humans; Isoleucine; Leucine; Propionic Acidemia; Valine; Vitamin B 12 Deficiency	2018
Amino acid metabolism in patients with propionic acidaemia. Journal of inherited metabolic disease, 2012, Volume: 35, Issue:1 Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids, Branched-Chain; Ammonia; Citric Acid Cycle; Diet; Glutamine; Glycine; Humans; Isoleucine; Lysine; Methylmalonyl-CoA Decarboxylase; Models, Biological; Propionic Acidemia; Urea; Valine	2012

Article

Year

Effects of medical food leucine content in the management of methylmalonic and propionic acidemias.

Current opinion in clinical nutrition and metabolic care, 2018, Volume: 21, Issue:1

Topics: Amino Acid Metabolism, Inborn Errors; Animals; Deficiency Diseases; Diet, Protein-Restricted; Foods, Specialized; Homocystinuria; Humans; Isoleucine; Leucine; Propionic Acidemia; Valine; Vitamin B 12 Deficiency

2018

Amino acid metabolism in patients with propionic acidaemia.

Journal of inherited metabolic disease, 2012, Volume: 35, Issue:1

Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids, Branched-Chain; Ammonia; Citric Acid Cycle; Diet; Glutamine; Glycine; Humans; Isoleucine; Lysine; Methylmalonyl-CoA Decarboxylase; Models, Biological; Propionic Acidemia; Urea; Valine

2012

Other Studies

2 other study(ies) available for valine and Propionic Acidemia

Article	Year
Acyl-CoA dehydrogenase substrate promiscuity: Challenges and opportunities for development of substrate reduction therapy in disorders of valine and isoleucine metabolism. Journal of inherited metabolic disease, 2023, Volume: 46, Issue:5 Topics: 2-Methyl-4-chlorophenoxyacetic Acid; Acyl-CoA Dehydrogenase; Carnitine; HEK293 Cells; Humans; Isoleucine; Propionic Acidemia; Valine	2023
Recapitulation of metabolic defects in a model of propionic acidemia using patient-derived primary hepatocytes. Molecular genetics and metabolism, 2016, Volume: 117, Issue:3 Topics: Actins; Amino Acids, Branched-Chain; Ammonia; Carbon-Carbon Ligases; Cells, Cultured; Child; Fibroblasts; Hemodynamics; Hepatocytes; Humans; Isoleucine; Liver; Methylmalonyl-CoA Decarboxylase; Mutation; Propionic Acidemia; Urea; Valine	2016

Article

Year

Acyl-CoA dehydrogenase substrate promiscuity: Challenges and opportunities for development of substrate reduction therapy in disorders of valine and isoleucine metabolism.

Journal of inherited metabolic disease, 2023, Volume: 46, Issue:5

Topics: 2-Methyl-4-chlorophenoxyacetic Acid; Acyl-CoA Dehydrogenase; Carnitine; HEK293 Cells; Humans; Isoleucine; Propionic Acidemia; Valine

2023

Recapitulation of metabolic defects in a model of propionic acidemia using patient-derived primary hepatocytes.

Molecular genetics and metabolism, 2016, Volume: 117, Issue:3

Topics: Actins; Amino Acids, Branched-Chain; Ammonia; Carbon-Carbon Ligases; Cells, Cultured; Child; Fibroblasts; Hemodynamics; Hepatocytes; Humans; Isoleucine; Liver; Methylmalonyl-CoA Decarboxylase; Mutation; Propionic Acidemia; Urea; Valine

2016