Remote Residues Affect Stability of Ornithine Transcarbamylase

The FASEB Journal ◽

10.1096/fasebj.29.1_supplement.572.29 ◽

2015 ◽

Vol 29 (S1) ◽

Author(s):

Kevin Ramos ◽

Lisa Ngu ◽

Nicholas DeLateur ◽

Mary Ondrechen ◽

Penny Beuning

Keyword(s):

Ornithine Transcarbamylase ◽

Remote Residues

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Differenzial diagnosis of acute encephalopathy: Acute decompensation in ornithine transcarbamylase (OTC) deficiency in a seven year old girl

Neuropediatrics ◽

10.1055/s-0031-1274047 ◽

2011 ◽

Vol 42 (S 01) ◽

Author(s):

C Abels ◽

B Fiedler ◽

W Weimer ◽

F Rutsch ◽

G Kurlemann

Keyword(s):

Ornithine Transcarbamylase ◽

Acute Encephalopathy ◽

Acute Decompensation ◽

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The Diagnosis and Management of Ornithine Transcarbamylase Deficiency in Pregnancy: A Case Report

Perinatal Journal ◽

10.2399/prn.11.0191006 ◽

2011 ◽

pp. 23-27

Author(s):

Orkun Çetin

Keyword(s):

Case Report ◽

Ornithine Transcarbamylase ◽

Ornithine Transcarbamylase Deficiency ◽

Diagnosis And Management ◽

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Ornithine transcarbamylase deficiency: Neuropathologic changes acquired in utero

The Journal of Pediatrics ◽

10.1016/s0022-3476(86)80935-0 ◽

1986 ◽

Vol 108 (6) ◽

pp. 942-945 ◽

Author(s):

F. Filloux ◽

J.J. Townsend ◽

C. Leonard

Keyword(s):

In Utero ◽

Ornithine Transcarbamylase ◽

Ornithine Transcarbamylase Deficiency

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GroEL, GroES, and ATP-dependent folding and spontaneous assembly of ornithine transcarbamylase.

Journal of Biological Chemistry ◽

10.1016/s0021-9258(18)53201-4 ◽

1993 ◽

Vol 268 (10) ◽

pp. 7489-7493 ◽

Author(s):

X. Zheng ◽

L.E. Rosenberg ◽

F. Kalousek ◽

W.A. Fenton

Keyword(s):

Ornithine Transcarbamylase

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Channeling of extramitochondrial ornithine to matrix ornithine transcarbamylase.

Journal of Biological Chemistry ◽

10.1016/s0021-9258(19)75910-9 ◽

1987 ◽

Vol 262 (1) ◽

pp. 203-208

Author(s):

N S Cohen ◽

C W Cheung ◽

L Raijman

Keyword(s):

Ornithine Transcarbamylase

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Pre-ornithine transcarbamylase. Properties of the cytoplasmic precursor of a mitochondrial matrix enzyme.

Journal of Biological Chemistry ◽

10.1016/s0021-9258(19)68500-5 ◽

1981 ◽

Vol 256 (21) ◽

pp. 10739-10742

Author(s):

J.P. Kraus ◽

J.G. Conboy ◽

L.E. Rosenberg

Keyword(s):

Ornithine Transcarbamylase ◽

Mitochondrial Matrix ◽

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Human ornithine transcarbamylase. Purification and characterization of the enzyme from normal liver and the liver of a Reye's syndrome patient.

Journal of Biological Chemistry ◽

10.1016/s0021-9258(17)39981-7 ◽

1977 ◽

Vol 252 (18) ◽

pp. 6464-6469 ◽

Author(s):

D L Pierson ◽

S L Cox ◽

B E Gilbert

Keyword(s):

Normal Liver ◽

Ornithine Transcarbamylase ◽

Syndrome Patient ◽

Reye's Syndrome ◽

Purification And Characterization

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Ornithine Transcarbamylase from Streptococcus faecalis and Bovine Liver

Journal of Biological Chemistry ◽

10.1016/s0021-9258(19)45528-2 ◽

1972 ◽

Vol 247 (6) ◽

pp. 1641-1653

Author(s):

Margaret Marshall ◽

Philip P. Cohen

Keyword(s):

Bovine Liver ◽

Ornithine Transcarbamylase ◽

Streptococcus Faecalis

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Uptake and processing of the precursor for rat liver ornithine transcarbamylase by isolated mitochondria. Inhibition by uncouplers.

Journal of Biological Chemistry ◽

10.1016/s0021-9258(19)68834-4 ◽

1981 ◽

Vol 256 (16) ◽

pp. 8263-8266 ◽

Author(s):

M. Mori ◽

T. Morita ◽

S. Miura ◽

M. Tatibana

Keyword(s):

Rat Liver ◽

Ornithine Transcarbamylase ◽

Isolated Mitochondria

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1H MRS allows brain phenotype differentiation in sisters with late onset ornithine transcarbamylase deficiency (OTCD) and discordant clinical presentations

Molecular Genetics and Metabolism ◽

10.1016/j.ymgme.2007.12.008 ◽

2008 ◽

Vol 94 (1) ◽

pp. 52-60 ◽

Author(s):

Andrea L. Gropman ◽

Rebecca R. Seltzer ◽

Marc Yudkoff ◽

Alice Sawyer ◽

John vanMeter ◽

...

Keyword(s):

Ornithine Transcarbamylase ◽

Ornithine Transcarbamylase Deficiency ◽

1H Mrs ◽

Clinical Presentations

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