Ornithine transcarbamylase deficiency: Pathogenesis of the cerebral disorder and new prospects for therapy

No abstract is available for this paper.

• Publication year

  1997

• Venue

  Metabolic brain disease

• Publication date

  1997-09-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1007/BF02674610PMID 9346466
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Urea cycle enzymes

  2001cited by this paper
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• Prolonged Metabolic Correction in Adult Ornithine Transcarbamylase-deficient Mice with Adenoviral Vectors (*)

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• Histological improvement in native liver after auxiliary partial liver transplantation for ornithine transcarbamylase deficiency.

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• Relative frequency of mutations causing ornithine transcarbamylase deficiency in 78 families

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• Loss of [3H]MK801 binding sites in brain in congenital ornithine transcarbamylase deficiency

  1995cited by this paper
• Tissue acylcarnitine and acyl-coenzyme A profiles in chronically hyperammonemic mice treated with sodium benzoate and supplementary L-carnitine.

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• Prospects for gene therapy in ornithine carbamoyltransferase deficiency and other urea cycle disorders

  1995cited by this paper
• Evidence of excitotoxicity in the brain of the ornithine carbamoyltransferase deficient sparse fur mouse.

  1995cited by this paper
• Orthotopic liver transplantation for ornithine transcarbamylase deficiency with hyperammonemic encephalopathy.

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  1995cited by this paper
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  1995cited by this paper
• Evidence for cholinergic neuronal loss in brain in congenital ornithine transcarbamylase deficiency.

  1994cited by this paper
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  1994cited by this paper
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• Orthotopic liver transplantation for urea cycle enzyme deficiency

  1992influential reference
• Application of reduced-size liver transplants as split grafts, auxiliary orthotopic grafts, and living related segmental transplants Broelsch CE, Emond JC, Whitington PF, Thistlethwaite JR, Baker AL, Lichtor JL. Ann Surg 1990;212:368–375

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  1990cited by this paper
• Increased densities of peripheral‐type benzodiazepine receptors in brain autopsy samples from cirrhotic patients with hepatic encephalopathy

  1990cited by this paper
• Studies of Central and “Peripheral-Type” Benzodiazepine Receptors in Autopsied Brain Tissue from Cirrhotic Patients with Hepatic Encephalopathy

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  1988cited by this paper
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  1986cited by this paper
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  1986cited by this paper
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  1986cited by this paper
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  1985cited by this paper
• Regulation of Acetylcholine Synthesis in the Brain

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  1984cited by this paper
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  1984cited by this paper
• Increase of Tryptophan and 5-Hydroxyindole Acetic Acid in the Brain of Ornithine Carbamoyltransferase Deficient Sparse-Fur Mice

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  1983cited by this paper
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  1983cited by this paper
• New pathways of nitrogen excretion in inborn errors of urea synthesis.

  1979cited by this paper
• Abnormal ornithine carbamoyltransferase in mice having the sparse-fur mutation.

  1976cited by this paper
• Congenital disorders of the urea cycle and ammonia detoxication.

  1971cited by this paper

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