Structure, function and assembly of mammalian branched-chain α-ketoacid dehydrogenase complex

1996 ◽  
pp. 101-117 ◽  
Author(s):  
R. M. Wynn ◽  
J. R. Davie ◽  
M. Meng ◽  
D. T. Chuang
Keyword(s):  
Structure Function ◽  
Branched Chain ◽  
Ketoacid Dehydrogenase ◽  
Dehydrogenase Complex

Effect of Dietary Protein on the Liver Content and Subunit Composition of the Branched-Chain α-Ketoacid Dehydrogenase Complex

1994 ◽  
Vol 308 (2) ◽  
pp. 446-453 ◽  
Author(s):  
Y. Zhao ◽  
K.M. Popov ◽  
Y. Shimomura ◽  
N.Y. Kedishvili ◽  
J. Jaskiewicz ◽  
...  
Keyword(s):  
Dietary Protein ◽  
Subunit Composition ◽  
Branched Chain ◽  
Ketoacid Dehydrogenase ◽  
Dehydrogenase Complex

Adverse effect of fenofibrate on branched-chain α-ketoacid dehydrogenase complex in rat's liver

Toxicology ◽  
2009 ◽  
Vol 266 (1-3) ◽  
pp. 1-5 ◽  
Author(s):  
Malgorzata Knapik-Czajka ◽  
Anna Gozdzialska ◽  
Jerzy Jaskiewicz
Keyword(s):  
Adverse Effect ◽  
Branched Chain ◽  
Ketoacid Dehydrogenase ◽  
Dehydrogenase Complex

Purification of branched chain α-ketoacid dehydrogenase complex from rat liver

1987 ◽  
Vol 163 (1) ◽  
pp. 74-78 ◽  
Author(s):  
Yoshiharu Shimomura ◽  
Ralph Paxton ◽  
Takayuki Ozawa ◽  
Robert A. Harris
Keyword(s):  
Rat Liver ◽  
Branched Chain ◽  
Ketoacid Dehydrogenase ◽  
Dehydrogenase Complex

scholarly journals Maple Syrup Urine Disease in a Central Indiana Hereford Herd

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Mark E. Robarge ◽  
Jonathan E. Beever ◽  
Stephen D. Lenz ◽  
Christopher J. Lynch ◽  
William L. Wigle
Keyword(s):  
Maple Syrup Urine Disease ◽  
Index Case ◽  
Clinical Signs ◽  
Maple Syrup ◽  
Hereford Cattle ◽  
Urine Disease ◽  
Branched Chain ◽  
Ketoacid Dehydrogenase ◽  
Dehydrogenase Complex ◽  
T Haplotype

Maple syrup urine disease (MSUD) and further cases were identified in herd mates of a small Hereford herd in Indiana based on history, clinical signs, microscopic lesions, and biochemical and genetic testing. This aminoacidopathy has been diagnosed in polled Shorthorn, polled Hereford, and Hereford cattle in Australia, Uruguay, Argentina, and Canada and is the result of a mutation of the branched-chain alpha-ketoacid dehydrogenase complex. The Indiana index calf case was confirmed by showing the classic accumulation of ketoacids in liver that results from a defect in the E1-alpha subunit (248 C/T haplotype) in the mitochondrial branched-chainα-ketoacid dehydrogenase complex. The presence of the mutation was confirmed in the index case, the dam, and four related herd mates that represent the first confirmed cases of bovine MSUD mutation in United States cattle.

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