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Disorders of Small Peptides

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Inborn Metabolic Diseases
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Abstract

This chapter deals with disorders in the catabolism of the dipeptides carnosine (carnosinemia) and homocarnosine (homocarnosinosis) and of the imidodipeptides (imidodipeptiduria, prolidase or peptidase D deficiency) Diseases due to defects in the metabolism of the tripeptide glutathione are discussed in Larsson (this volume).

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References

  1. Perry TL, Hansen S, Tischler B, Bunting R, Berry K (1967) Carnosinemia: a new metabolic disorder associated with neurological disease and mental defect. N Engl J Med 277: 1219–1227

    Article  PubMed  CAS  Google Scholar 

  2. Cohen M, Hartlage PL, Krawiecki N, Roesel RA, Carter AL, Hommes FA (1985) Serum carnosinase deficiency: a non-disabling phenotype? J Ment Defic, Res 29: 383–389

    Google Scholar 

  3. Sjaastadt O, Berstadt J, Gjesdahl P, Gjessing L(1976) Homocarnosinosis. 2. A familial metabolic

    Google Scholar 

  4. disorder associated with spastic paraplegia, progres- sive mental deficiency, and retinal pigmentation.

    Google Scholar 

  5. Acta Neurol Scand 53: 275–290

    Google Scholar 

  6. Lenney JF, Peppers SC, Kucera CM, Sjaastadt O(1983) Homocarnosinosis: lack of serum carnosinase is the defect probably responsible for elevated and CSF homocarnosine. Clin Chim Acta 132: 157–165

    Google Scholar 

  7. Goodman SI, Solomons CC, Muschenheim F, Macintyre CA, Miles B, O’Brien D (1968) A syndrome resembling lathyrism associated with iminodipeptiduria. Am J Med 45: 152–159

    Article  PubMed  CAS  Google Scholar 

  8. Larregue M, Charpentier C, Laidet B, Lambert M, Bressieux J-M (1982) Déficit en prolidase

    Google Scholar 

  9. Arata J, Hatakenaka K, Oono T (1986) Effect of topical application of glycine and proline on recalci trant leg ulcers of prolidase deficiency. Arch Dermatol 122: 626–627

    Article  PubMed  CAS  Google Scholar 

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© 1995 Springer-Verlag Berlin Heidelberg

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Jaeken, J. (1995). Disorders of Small Peptides. In: Fernandes, J., Saudubray, JM., Van den Berghe, G., Tada, K., Buist, N.R.M. (eds) Inborn Metabolic Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-03147-6_19

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  • DOI: https://doi.org/10.1007/978-3-662-03147-6_19

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-03149-0

  • Online ISBN: 978-3-662-03147-6

  • eBook Packages: Springer Book Archive

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