D J Danner|C B Doering - JoVE Visualize

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Biochimica Et Biophysica Acta|December 31, 1997

Two new mutations in the human E1 beta subunit of branched chain alpha-ketoacid dehydrogenase associated with maple syrup urine diseaseB B McConnell, B Burkholder, D J Danner

The Journal of Biological Chemistry|September 5, 1989

Reversion of the maple syrup urine disease phenotype of impaired branched chain alpha-ketoacid dehydrogenase complex activity in fibroblasts from an affected childS Litwer, W J Herring, D J Danner

Journal of Cellular Biochemistry|April 1, 1996

Influence of subunit transcript and protein levels on formation of a mitochondrial multienzyme complexB B McConnell, M C McKean, D J Danner

The Journal of Biological Chemistry|January 25, 1982

Clofibric acid and phenylpyruvic acid as biochemical probes for studying soluble bovine liver branched chain ketoacid dehydrogenaseD J Danner, E T Sewell, L J Elsas

Human Molecular Genetics|October 1, 1996

Functional analysis in Saccharomyces cerevisiae of naturally occurring amino acid substitutions in human dihydrolipoamide dehydrogenaseM M Lanterman, J R Dickinson, D J Danner

The Biochemical Journal|August 1, 1983

Antibodies to bovine liver branched-chain 2-oxo acid dehydrogenase cross-react with this enzyme complex from other tissues and speciesS C Heffelfinger, E T Sewell, D J Danner

Biochemistry|November 22, 1983

Identification of specific subunits of highly purified bovine liver branched-chain ketoacid dehydrogenaseS C Heffelfinger, E T Sewell, D J Danner

Biochimica Et Biophysica Acta|November 15, 1992

Nucleotide sequence of the 5' end including the initiation codon of cDNA for the E1 alpha subunit of the human branched chain alpha-ketoacid dehydrogenase complexM C McKean, K A Winkeler, D J Danner

Gene Therapy|September 22, 2012

Generation of an optimized lentiviral vector encoding a high-expression factor VIII transgene for gene therapy of hemophilia AJ M Johnston, G Denning, C B Doering, et al.

Biochimica Et Biophysica Acta|March 20, 1992

Branched chain acyltransferase absence due to an Alu-based genomic deletion allele and an exon skipping allele in a compound heterozygote proband expressing maple syrup urine diseaseW J Herring, M McKean, N Dracopoli, et al.

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Showing results (11-20 of 45) with videos related to

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Pageof 5

Biochimica Et Biophysica Acta|December 31, 1997

Two new mutations in the human E1 beta subunit of branched chain alpha-ketoacid dehydrogenase associated with maple syrup urine diseaseB B McConnell, B Burkholder, D J Danner

The Journal of Biological Chemistry|September 5, 1989

Reversion of the maple syrup urine disease phenotype of impaired branched chain alpha-ketoacid dehydrogenase complex activity in fibroblasts from an affected childS Litwer, W J Herring, D J Danner

Journal of Cellular Biochemistry|April 1, 1996

Influence of subunit transcript and protein levels on formation of a mitochondrial multienzyme complexB B McConnell, M C McKean, D J Danner

The Journal of Biological Chemistry|January 25, 1982

Clofibric acid and phenylpyruvic acid as biochemical probes for studying soluble bovine liver branched chain ketoacid dehydrogenaseD J Danner, E T Sewell, L J Elsas

Human Molecular Genetics|October 1, 1996

Functional analysis in Saccharomyces cerevisiae of naturally occurring amino acid substitutions in human dihydrolipoamide dehydrogenaseM M Lanterman, J R Dickinson, D J Danner

The Biochemical Journal|August 1, 1983

Antibodies to bovine liver branched-chain 2-oxo acid dehydrogenase cross-react with this enzyme complex from other tissues and speciesS C Heffelfinger, E T Sewell, D J Danner

Biochemistry|November 22, 1983

Identification of specific subunits of highly purified bovine liver branched-chain ketoacid dehydrogenaseS C Heffelfinger, E T Sewell, D J Danner

Biochimica Et Biophysica Acta|November 15, 1992

Nucleotide sequence of the 5' end including the initiation codon of cDNA for the E1 alpha subunit of the human branched chain alpha-ketoacid dehydrogenase complexM C McKean, K A Winkeler, D J Danner

Gene Therapy|September 22, 2012

Generation of an optimized lentiviral vector encoding a high-expression factor VIII transgene for gene therapy of hemophilia AJ M Johnston, G Denning, C B Doering, et al.

Biochimica Et Biophysica Acta|March 20, 1992

Branched chain acyltransferase absence due to an Alu-based genomic deletion allele and an exon skipping allele in a compound heterozygote proband expressing maple syrup urine diseaseW J Herring, M McKean, N Dracopoli, et al.

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