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PMID: 1826833 Published · ppublish English Journal Article

Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis).

The Biochemical journal ·Vol. 275 ( Pt 1) ·1991-04-01 ·Pages 269-72

Hall NA, Lake BD, Dewji NN, Patrick AD

Abstract

Immunochemical studies demonstrate that subunit c of mitochondrial ATP synthase is stored in the late-infantile, juvenile and adult forms of Batten's disease. It does not accumulate in the infantile form, or in other conditions involving lysosomal hypertrophy. These results suggest that the defective metabolism of subunit c is central to the pathogenesis of these three forms of Batten's disease.

MeSH Terms
Aged Aging/metabolism Blotting, Western Brain/enzymology,ultrastructure Humans Immunoenzyme Techniques Lysosomes/enzymology Macromolecular Substances Middle Aged Mitochondria/enzymology Molecular Weight Neuronal Ceroid-Lipofuscinoses/enzymology Proton-Translocating ATPases/metabolism Submitochondrial Particles/enzymology
Chemicals
Macromolecular Substances Proton-Translocating ATPases
Authors & Affiliations
4 authors, click to expand affiliations / ORCID
Hall N A
Department of Clinical Biochemistry, Institute of Child Health, London, U.K.
Lake B D
Dewji N N
Patrick A D
References (16)
16 references, click to expand
  1. Glycoconjugates in storage cytosomes from ceroid-lipofuscinosis (Batten's disease) and in lipofuscin from old-age brain.
    Adv Exp Med Biol. 1989;266:225-41; discussion 242 PMID: 2486152
  2. Cleavage of structural proteins during the assembly of the head of bacteriophage T4.
    Nature. 1970 Aug 15;227(5259):680-5 PMID: 5432063
  3. Infantile form of neuronal ceroid lipofuscinosis (CLN1) maps to the short arm of chromosome 1.
    Genomics. 1991 Jan;9(1):170-3 PMID: 1672288
  4. Batten disease (Spielmeyer-Vogt disease, juvenile onset neuronal ceroid-lipofuscinosis) gene (CLN3) maps to human chromosome 16.
    Genomics. 1990 Oct;8(2):387-90 PMID: 2249854
  5. Kufs' disease: a critical reappraisal.
    Brain. 1988 Feb;111 ( Pt 1):27-62 PMID: 3284607
  6. Neuronal ceroid-lipofuscinoses in childhood.
    Brain Dev. 1988;10(2):80-3 PMID: 3291628
  7. Isolation and characterisation of monoclonal antibodies against hydrophobic membrane subunit 9 of the yeast mitochondrial H+-ATPase.
    Biochim Biophys Acta. 1988 Mar 30;933(1):223-8 PMID: 2450580
  8. Ovine ceroid lipofuscinosis. The major lipopigment protein and the lipid-binding subunit of mitochondrial ATP synthase have the same NH2-terminal sequence.
    J Biol Chem. 1989 Apr 5;264(10):5736-40 PMID: 2522438
  9. High-mannose dolichol-linked oligosaccharides in infantile ceroid-lipofuscinosis.
    Biochem Soc Trans. 1989 Dec;17(6):1032-3 PMID: 2628062
  10. Analysis of dolichyl pyrophosphoryl oligosaccharides in purified storage cytosomes from ovine ceroid-lipofuscinosis.
    Biochim Biophys Acta. 1989 Dec 8;993(2-3):245-51 PMID: 2597696
  11. Ceroid lipofuscinosis in sheep. II. The major component of the lipopigment in liver, kidney, pancreas, and brain is low molecular weight protein.
    J Biol Chem. 1986 Feb 5;261(4):1773-7 PMID: 3944108
  12. The sequence of the major protein stored in ovine ceroid lipofuscinosis is identical with that of the dicyclohexylcarbodiimide-reactive proteolipid of mitochondrial ATP synthase.
    Biochem J. 1990 Jun 15;268(3):751-8 PMID: 2141977
  13. Morphologic studies on adult neuronal-ceroid lipofuscinosis (NCL).
    Clin Neuropathol. 1982;1(4):151-62 PMID: 7168921
  14. Isolation of carbodiimide-binding proteins from mitochondria and Escherichia coli.
    Methods Enzymol. 1979;55:426-34 PMID: 156852
  15. Purification of the carbodiimide-reactive protein component of the ATP energy-transducing system of Escherichia coli.
    J Biol Chem. 1976 Nov 10;251(21):6630-7 PMID: 789371
  16. Lysosomal storage of the DCCD reactive proteolipid subunit of mitochondrial ATP synthase in human and ovine ceroid lipofuscinoses.
    Adv Exp Med Biol. 1989;266:211-22; discussion 223 PMID: 2535017
Article Info
Journal
The Biochemical journal
Abbr.
Biochem J
ISSN
0264-6021
DOI
10.1042/bj2750269
Published
1991-04-01
Pages
269-72
Language
English
Region
England
NLM ID
2984726R
PMCID
PMC1150044
Subset
IM
External Links
PubMed source DOI: 10.1042/bj2750269 PMC Full Text View journal details
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