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Propionic acidemia

Known as: Propionyl-CoA carboxylase deficiency, Hyperglycinemia With Ketoacidosis And Leukopenia, Propionic Acidemia [Disease/Finding] 
Autosomal recessive metabolic disorder caused by mutations in PROPIONYL-COA CARBOXYLASE genes that result in dysfunction of branch chain amino acids… 
National Institutes of Health

Related topics

Related topics

37 relations
Acidosis, LacticAcute encephalopathyAnemiaApnea

Narrower (2)

CoQ-responsive OXPHOS deficiencyPropionicaciduria

Broader (2)

Enzyme DeficiencyHyperglycinemia

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
1986
1986
Coding sequence of the precursor of the beta subunit of rat propionyl-CoA carboxylase.
A cDNA encoding the cytoplasmic precursor of the beta subunit of the mitochondrial enzyme propionyl-CoA carboxylase (EC 6.4.1.3… 
Highly Cited
1985
Highly Cited
1985
Bonding of restorative resins to dentine promoted by aqueous mixtures of aldehydes and active monomers.
Effective bonding between restorative resins and hard dental tissues would eliminate the need for retentive undercuts and prevent… 
Highly Cited
1982
Highly Cited
1982
Purification of low-abundance messenger RNAs from rat liver by polysome immunoadsorption.
We have purified three low-abundance hepatic mRNAs to near homogeneity by polysome immunoadsorption. The mRNAs coding for the… 
1982
1982
CYSTINOTIC AND NORMAL FIBROBLASTS: DIFFERENTIAL PROTECTION IN CYSTINE-FREE MEDIUM BY DITHIOTHREITOL
Summary: We studied survival of normal (n=4) and cystinotic (n=3) fibroblast strains in selective (cystine-free) medium, with or… 
1981
1981
Relationship of metabolic activation of N-hydroxy-N-acylarylamines to biological response in the liver and mammary gland of the female CD rat.
Intraperitoneal injection of the N-formyl, N-acetyl, or N-propionyl derivatives of N-hydroxy-4-aminobiphenyl, N-hydroxy-2… 
1980
1980
Metabolic regulation in tylosin-producing Streptomyces fradiae: regulatory role of adenylate nucleotide pool and enzymes involved in biosynthesis of tylonolide precursors
The relationship was studied between the level of the intracellular adenylates and the biosynthesis of tylosin by Streptomyces… 
1976
1976
Magnetic resonance studies of the proximity and spatial arrangement of propionyl coenzyme A and pyruvate on a biotin-metalloenzyme, transcarboxylase.
A spin-labeled ester of CoA, R-CoA (3-carboxy-2,2,5,5-tetramethyl-1-pyrolidinyl-1-oxy CoA thioester), has been shown by… 
1962
1962
HEPATIC DYSFUNCTION ASSOCIATED WITH TRIACETYLOLEANDOMYCIN AND PROPIONYL ERYTHROMYCIN ESTER LAURYL SULFATE
Highly Cited
1961
Highly Cited
1961
Metabolism of propionic acid in animal tissues. VIII. Crystalline propionyl carboxylase.
Highly Cited
1959
Highly Cited
1959
Metabolism of propionic acid in animal tissues. V. Purification and properties of propionyl carboxylase.
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