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  • 1
    Electronic Resource
    Electronic Resource
    End-stage liver disease as the only consequence of a mitochondrial respiratory chain deficiency: no contra-indication for liver transplantation (2000)
    Springer
    European journal of pediatrics 159 (2000), S. 523-526 
    Details
    ISSN: 1432-1076
    Keywords: Key words End-stage liver disease ; Liver transplantation ; Inborn errors of metabolism ; Mitochondrial respiratory chain deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The prerequisite for liver transplantation as a therapeutic option for inherited metabolic diseases should be that the enzyme defect, being responsible for the major clinical (hepatic and/or extra-hepatic) abnormalities, is localised in the liver. Furthermore, no adequate dietary or pharmacological treatment should be available or such treatment should have an unacceptable influence on the quality of life. We report an infant, who developed end-stage liver disease with persistent lactic acidaemia in his first months of life. Analysis of the mitochondrial respiratory chain in liver tissue revealed a combined partial complex I and IV deficiency. No extra-hepatic involvement could be demonstrated by careful screening for multiple organ involvement, including analysis of the mitochondrial respiratory chain in muscle tissue and cultured skin fibroblasts. The boy received a reduced size liver graft at the age of 8 months. He recovered successfully. Almost 5 years after transplantation he is in good clinical condition. No clinical or biochemical signs of any organ dysfunction have been demonstrated. The considerations on which basis it was decided that there was no contra-indication to perform liver transplantation in this patient are discussed. Conclusion The possibility of a mitochondrial respiratory chain deficiency should be considered in liver disease of unknown origin prior to liver transplantation. Liver transplantation is a therapeutic option in mitochondrial respiratory chain deficiency-based end-stage liver disease provided that extra-hepatic involvement is carefully excluded.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310051324
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  • 2
    Electronic Resource
    Electronic Resource
    D-Amino Acids as Putative Neurotransmitters: Focus on D-Serine (2000)
    Springer
    Neurochemical research 25 (2000), S. 553-560 
    Details
    ISSN: 1573-6903
    Keywords: D-serine ; D-aspartate ; astrocytes ; N-methyl-D-aspartate (NMDA) receptor ; Bergmann glia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Of the twenty amino acids in the mammalian body, only serine and aspartate occur in D-configuration as well as L-configuration in significant amount. D-serine is selectively concentrated in the brain, localized to protoplasmic astrocytes that ensheath synapses and distributed similarly to N-methyl-D-aspartate (NMDA) subtype of glutamate receptors. D-serine has been found to function as an endogenous ligand for the “glycine” site of the NMDA receptor. Evidences for this include the greater potency of D-serine to activate this site than glycine, and D-amino acid oxidase, which degrades D-serine as well as other neutral D-amino acids, markedly attenuates NMDA neurotransmission. D-serine is also formed by serine racemase, a recently cloned enzyme that converts L-serine to D-serine. Thus, in many ways D-serine fulfills criteria for defining its functionality as a neurotransmitter and challenges the dogma relating to neurotransmission, for it is the “unnatural” isomeric form of an amino acid derived from glia rather than neurons.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1023/A:1007586314648
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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