Molecular Genetic Basis for Inherited Human Disorders of Branched-Chain ?-Keto Acid Dehydrogenase Complex

Annals of the New York Academy of Sciences ◽

10.1111/j.1749-6632.1989.tb15012.x ◽

1989 ◽

Vol 573 (1 Alpha-Keto Ac) ◽

pp. 369-377 ◽

Author(s):

DEAN J. DANNER ◽

STUART LITWER ◽

W. JOSEPH HERRING ◽

LOUIS J. ELSAS

Keyword(s):

Genetic Basis ◽

Molecular Genetic ◽

Keto Acid ◽

Acid Dehydrogenase ◽

Molecular Genetic Basis ◽

Human Disorders ◽

Branched Chain ◽

Acid Dehydrogenase Complex ◽

Dehydrogenase Complex

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Characterization of the promoter-regulatory region and structural organization of E1 alpha gene (BCKDHA) of human branched-chain alpha-keto acid dehydrogenase complex.

Journal of Biological Chemistry ◽

10.1016/s0021-9258(18)53097-0 ◽

1993 ◽

Vol 268 (11) ◽

pp. 8309-8316

Author(s):

J.L. Chuang ◽

R.P. Cox ◽

D.T. Chuang

Keyword(s):

Structural Organization ◽

Regulatory Region ◽

Keto Acid ◽

Acid Dehydrogenase ◽

Branched Chain ◽

Acid Dehydrogenase Complex ◽

Dehydrogenase Complex

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Subunit structure of the dihydrolipoyl transacylase component of branched-chain alpha-keto acid dehydrogenase complex from bovine liver. Mapping of the lipoyl-bearing domain by limited proteolysis.

Journal of Biological Chemistry ◽

10.1016/s0021-9258(17)42477-x ◽

1986 ◽

Vol 261 (1) ◽

pp. 343-349 ◽

Author(s):

C W Hu ◽

T A Griffin ◽

K S Lau ◽

R P Cox ◽

D T Chuang

Keyword(s):

Limited Proteolysis ◽

Bovine Liver ◽

Subunit Structure ◽

Keto Acid ◽

Acid Dehydrogenase ◽

Liver Mapping ◽

Branched Chain ◽

Acid Dehydrogenase Complex ◽

Dehydrogenase Complex

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Cloning and expression in Escherichia coli of mature E1 beta subunit of bovine mitochondrial branched-chain alpha-keto acid dehydrogenase complex. Mapping of the E1 beta-binding region on E2.

Journal of Biological Chemistry ◽

10.1016/s0021-9258(18)46029-2 ◽

1992 ◽

Vol 267 (3) ◽

pp. 1881-1887

Author(s):

R M Wynn ◽

J L Chuang ◽

J R Davie ◽

C W Fisher ◽

M A Hale ◽

...

Keyword(s):

Escherichia Coli ◽

Beta Subunit ◽

Cloning And Expression ◽

Keto Acid ◽

Binding Region ◽

Expression In Escherichia Coli ◽

Acid Dehydrogenase ◽

Branched Chain ◽

Acid Dehydrogenase Complex ◽

Dehydrogenase Complex

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Isolation and sequencing of a cDNA encoding the decarboxylase (E1)alpha precursor of bovine branched-chain alpha-keto acid dehydrogenase complex. Expression of E1 alpha mRNA and subunit in maple-syrup-urine-disease and 3T3-L1 cells.

Journal of Biological Chemistry ◽

10.1016/s0021-9258(18)68408-x ◽

1988 ◽

Vol 263 (18) ◽

pp. 9007-9014 ◽

Author(s):

C W Hu ◽

K S Lau ◽

T A Griffin ◽

J L Chuang ◽

C W Fisher ◽

...

Keyword(s):

Maple Syrup Urine Disease ◽

Keto Acid ◽

Maple Syrup ◽

Acid Dehydrogenase ◽

Urine Disease ◽

Branched Chain ◽

Acid Dehydrogenase Complex ◽

Complex Expression ◽

Dehydrogenase Complex

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Hepatic Branched-Chain .ALPHA.-Keto Acid Dehydrogenase Complex in Female Rats: Activation by Exercise and Starvation.

Journal of Nutritional Science and Vitaminology ◽

10.3177/jnsv.45.303 ◽

1999 ◽

Vol 45 (3) ◽

pp. 303-309 ◽

Author(s):

Rumi KOBAYASHI ◽

Yoshiharu SHIMOMURA ◽

Taro MURAKAMI ◽

Naoya NAKAI ◽

Megumi OTSUKA ◽

...

Keyword(s):

Female Rats ◽

Keto Acid ◽

Acid Dehydrogenase ◽

Branched Chain ◽

Acid Dehydrogenase Complex ◽

Dehydrogenase Complex

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Lowered Concentrations of Branched-Chain Amino Acids Result in Impaired Growth and Neurological Problems: Insights from a Branched-Chain α-Keto Acid Dehydrogenase Complex Kinase-Deficient Mouse Model

Nutrition Reviews ◽

10.1111/j.1753-4887.2007.tb00296.x ◽

2008 ◽

Vol 65 (4) ◽

pp. 167-172

Author(s):

Malcolm Watford

Keyword(s):

Amino Acids ◽

Mouse Model ◽

Branched Chain Amino Acids ◽

Deficient Mouse ◽

Keto Acid ◽

Acid Dehydrogenase ◽

Branched Chain ◽

Acid Dehydrogenase Complex ◽

Dehydrogenase Complex

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Genetic reconstruction and characterization of the recombinant transacylase (E2b) component of bovine branched-chain alpha-keto acid dehydrogenase complex. Implication of histidine 391 as an active site residue.

Journal of Biological Chemistry ◽

10.1016/s0021-9258(19)38282-1 ◽

1990 ◽

Vol 265 (22) ◽

pp. 13174-13180

Author(s):

T A Griffin ◽

D T Chuang

Keyword(s):

Active Site ◽

Keto Acid ◽

Active Site Residue ◽

Acid Dehydrogenase ◽

Branched Chain ◽

Acid Dehydrogenase Complex ◽

Dehydrogenase Complex

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[39] Purification, resolution, and reconstitution of branched-chain 2-keto acid dehydrogenase complex from bovine kidney

Methods in Enzymology - Branched-Chain Amino Acids ◽

10.1016/s0076-6879(88)66041-1 ◽

1988 ◽

pp. 303-308 ◽

Author(s):

Kenneth G. Cook ◽

Stephen J. Yeaman

Keyword(s):

Keto Acid ◽

Bovine Kidney ◽

Acid Dehydrogenase ◽

Branched Chain ◽

Acid Dehydrogenase Complex ◽

Dehydrogenase Complex

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Regulation of branched-chain α-keto acid dehydrogenase complex in rat liver and skeletal muscle by exercise and nutrition

Alpha-Keto Acid Dehydrogenase Complexes ◽

10.1007/978-3-0348-8981-0_13 ◽

1996 ◽

pp. 177-186 ◽

Author(s):

Y. Shimomura

Keyword(s):

Skeletal Muscle ◽

Rat Liver ◽

Keto Acid ◽

Acid Dehydrogenase ◽

Branched Chain ◽

Acid Dehydrogenase Complex ◽

Dehydrogenase Complex

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Structure of the gene encoding the entire mature E1α subunit of human branched-chain α-keto acid dehydrogenase complex

FEBS Letters ◽

10.1016/0014-5793(91)80755-r ◽

1991 ◽

Vol 284 (1) ◽

pp. 34-38 ◽

Author(s):

Nahid Dariush ◽

Charles W. Fisher ◽

Rody P. Cox ◽

David T. Chuang

Keyword(s):

Keto Acid ◽

Gene Encoding ◽

Acid Dehydrogenase ◽

Branched Chain ◽

Acid Dehydrogenase Complex ◽

Dehydrogenase Complex

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