Literature DB >> 6124241

Copper metabolism in mottled mouse mutants. The effect of copper therapy on lysyl oxidase activity in brindled (Mobr) mice.

P M Royce, J Camakaris, J R Mann, D M Danks.   

Abstract

Lysyl oxidase activity in extracts of skin from 1-day-old Mobr/Y mice was found to be between 50 and 60% of that in corresponding extracts from littermate +/Y mice of the same age. It was increased to 84-150% of that in the latter by prior treatment of the Mobr/Y mice at 7 days of age with a single subcutaneous injection of 50 micrograms of copper, retained as Cu+ in an alkyl polyether/sebacic acid solution. This suggests that in this form the copper is able to by-pass the block in copper metabolism and is deliverable to copper-requiring processes.

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Year:  1982        PMID： 6124241      PMCID： PMC1158120          DOI： 10.1042/bj2020369

Source DB:  PubMed          Journal:  Biochem J        ISSN： 0264-6021            Impact factor:   3.857


  16 in total

1.  Induction of lysyl oxidase with copper. Properties of an in vitro system.

Authors:  J K Rayton; E D Harris
Journal:  J Biol Chem       Date:  1979-02-10       Impact factor: 5.157

2.  Reduced lysyl oxidase activity in skin fibroblasts from patients with Menkes' syndrome.

Authors:  P M Royce; J Camakaris; D M Danks
Journal:  Biochem J       Date:  1980-11-15       Impact factor: 3.857

3.  Impaired copper homeostasis in neonatal male and adult female Brindled (Mobr) mice.

Authors:  G W Evans; B L Reis
Journal:  J Nutr       Date:  1978-04       Impact factor: 4.798

4.  Decreased lysyl oxidase activity in the aneurysm-prone, mottled mouse.

Authors:  D W Rowe; E B McGoodwin; G R Martin; D Grahn
Journal:  J Biol Chem       Date:  1977-02-10       Impact factor: 5.157

5.  Copper metabolism in mottled mouse mutants: copper concentrations in tissues during development.

Authors:  J Camakaris; J R Mann; D M Danks
Journal:  Biochem J       Date:  1979-06-15       Impact factor: 3.857

6.  Copper metabolism in mottled mouse mutants: distribution of 64Cu in brindled (Mobr) mice.

Authors:  J R Mann; J Camakaris; D M Danks
Journal:  Biochem J       Date:  1979-06-15       Impact factor: 3.857

7.  Menkes's syndrome. Report of a patient treated from 21 days of age with parenteral copper.

Authors:  P Daish; E M Wheeler; P F Roberts; R D Jones
Journal:  Arch Dis Child       Date:  1978-12       Impact factor: 3.791

8.  Copper metabolism in mottled mouse mutants: copper therapy of brindled (Mobr) mice.

Authors:  J R Mann; J Camakaris; D M Danks; E G Walliczek
Journal:  Biochem J       Date:  1979-06-15       Impact factor: 3.857

9.  Menkes kinky hair syndrome: Is it a treatable disorder?

Authors:  A D Garnica; J L Frias; O M Rennert
Journal:  Clin Genet       Date:  1977-02       Impact factor: 4.438

10.  Menkes disease: a biochemical abnormality in cultured human fibroblasts.

Authors:  T J Goka; R E Stevenson; P M Hefferan; R R Howell
Journal:  Proc Natl Acad Sci U S A       Date:  1976-02       Impact factor: 11.205
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  10 in total

1.  Type IX Ehlers-Danlos syndrome and Menkes syndrome: the decrease in lysyl oxidase activity is associated with a corresponding deficiency in the enzyme protein.

Authors:  H Kuivaniemi; L Peltonen; K I Kivirikko
Journal:  Am J Hum Genet       Date:  1985-07       Impact factor: 11.025

Review 2.  ATP7A-related copper transport diseases-emerging concepts and future trends.

Authors:  Stephen G Kaler
Journal:  Nat Rev Neurol       Date:  2011-01       Impact factor: 42.937

Review 3.  Of mice and men, metals and mutations.

Authors:  D M Danks
Journal:  J Med Genet       Date:  1986-04       Impact factor: 6.318

4.  Comparisons of copper deficiency states in the murine mutants blotchy and brindled. Changes in copper-dependent enzyme activity in 13-day-old mice.

Authors:  M Phillips; J Camakaris; D M Danks
Journal:  Biochem J       Date:  1986-08-15       Impact factor: 3.857

5.  The relationship of excess copper accumulation by fibroblasts from the brindled mouse model of Menkes disease to the primary defect.

Authors:  G L Waldrop; M J Ettinger
Journal:  Biochem J       Date:  1990-04-15       Impact factor: 3.857

6.  Fibroblast silver loading for the diagnosis of Menkes disease.

Authors:  F W Verheijen; C E Beerens; A C Havelaar; W J Kleijer; G M Mancini
Journal:  J Med Genet       Date:  1998-10       Impact factor: 6.318

Review 7.  Mutations in humans and animals which affect copper metabolism.

Authors:  J Camakaris; M Phillips; D M Danks; R Brown; T Stevenson
Journal:  J Inherit Metab Dis       Date:  1983       Impact factor: 4.982

8.  Similar splicing mutations of the Menkes/mottled copper-transporting ATPase gene in occipital horn syndrome and the blotchy mouse.

Authors:  S Das; B Levinson; C Vulpe; S Whitney; J Gitschier; S Packman
Journal:  Am J Hum Genet       Date:  1995-03       Impact factor: 11.025

9.  Investigation of iron metabolism in mice expressing a mutant Menke's copper transporting ATPase (Atp7a) protein with diminished activity (Brindled; Mo (Br) (/y) ).

Authors:  Sukru Gulec; James F Collins
Journal:  PLoS One       Date:  2013-06-11       Impact factor: 3.240

Review 10.  Mottled Mice and Non-Mammalian Models of Menkes Disease.

Authors:  Małgorzata Lenartowicz; Wojciech Krzeptowski; Paweł Lipiński; Paweł Grzmil; Rafał Starzyński; Olga Pierzchała; Lisbeth Birk Møller
Journal:  Front Mol Neurosci       Date:  2015-12-18       Impact factor: 5.639
  10 in total

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