Genetic complementation of propionyl-CoA carboxylase deficiency in cultured human fibroblasts.
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The molecular basis of 3-methylcrotonylglycinuria, a disorder of leucine catabolismIsolation of cDNA clones coding for the alpha and beta chains of human propionyl-CoA carboxylase: chromosomal assignments and DNA polymorphisms associated with PCCA and PCCB genesHuman argininosuccinate lyase: a structural basis for intragenic complementationGenetics of propionic acidemia in a Mennonite-Amish kindredCoding sequence of the precursor of the beta subunit of rat propionyl-CoA carboxylaseAnalysis of genetic complementation by whole-cell microtechniques in fibroblast heterokaryonsGenetic heterogeneity among patients with methylcobalamin deficiency. Definition of two complementation groups, cblE and cblG.Treatment of a neonate with propionic acidaemia and severe hyperammonaemia by peritoneal dialysis.Cloning of functional alpha propionyl CoA carboxylase and correction of enzyme deficiency in pccA fibroblastsUnequal synthesis and differential degradation of propionyl CoA carboxylase subunits in cells from normal and propionic acidemia patients.Genetic complementation analysis of 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency in cultured fibroblasts.Biochemical characterization of biotin-responsive multiple carboxylase deficiency: heterogeneity within the bio genetic complementation group.Evidence for a defect of holocarboxylase synthetase activity in cultured lymphoblasts from a patient with biotin-responsive multiple carboxylase deficiency.The biotin-dependent carboxylase deficienciesGenetic complementation among inherited deficiencies of methylmalonyl-CoA mutase activity: evidence for a new class of human cobalamin mutant.Biochemical differences between mutant propionyl-CoA carboxylases from two complementation groups.Absence of cross-reacting material in isolated propionyl CoA carboxylase deficiency: nature of residual carboxylating activityKinetic analysis of genetic complementation in heterokaryons of propionyl CoA carboxylase-deficient human fibroblastsInfantile sialidosis: a phenocopy of type 1 GM1 gangliosidosis distinguished by genetic complementation and urinary oligosaccharides.Mutations participating in interallelic complementation in propionic acidemiaInterallelic complementation in an inborn error of metabolism: genetic heterogeneity in argininosuccinate lyase deficiencyComplementation studies of isovaleric acidemia and glutaric aciduria type II using cultured skin fibroblasts.Biotin-response organicaciduria. Multiple carboxylase defects and complementation studies with propionicacidemia in cultured fibroblasts.Heterozygote expression in propionyl coenzyme A carboxylase deficiency. Differences between major complementation groupsBovine kidney 3-methylcrotonyl-CoA and propionyl-CoA carboxylases: each enzyme contains nonidentical subunits.An unusual insertion/deletion in the gene encoding the beta-subunit of propionyl-CoA carboxylase is a frequent mutation in Caucasian propionic acidemia.An introduction to gas chromatography-mass spectrometry and the inherited organic acidemias.Propionicacidemia: absence of alpha-chain mRNA in fibroblasts from patients of the pccA complementation group.Assignment of the alpha and beta chains of human propionyl-CoA carboxylase to genetic complementation groupsImport of TAT-conjugated propionyl-CoA carboxylase using models of propionic acidemia.Propionyl-CoA carboxylase - A review.
P2860
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P2860
Genetic complementation of propionyl-CoA carboxylase deficiency in cultured human fibroblasts.
description
1977 nî lūn-bûn
@nan
1977 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
1977 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
1977年の論文
@ja
1977年論文
@yue
1977年論文
@zh-hant
1977年論文
@zh-hk
1977年論文
@zh-mo
1977年論文
@zh-tw
1977年论文
@wuu
name
Genetic complementation of pro ...... in cultured human fibroblasts.
@ast
Genetic complementation of pro ...... in cultured human fibroblasts.
@en
type
label
Genetic complementation of pro ...... in cultured human fibroblasts.
@ast
Genetic complementation of pro ...... in cultured human fibroblasts.
@en
prefLabel
Genetic complementation of pro ...... in cultured human fibroblasts.
@ast
Genetic complementation of pro ...... in cultured human fibroblasts.
@en
P2093
P2860
P1476
Genetic complementation of pro ...... in cultured human fibroblasts.
@en
P2093
P2860
P304
P407
P577
1977-07-01T00:00:00Z