Literature DB >> 1826833

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

N A Hall1, B D Lake, N N Dewji, A D Patrick.   

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.

Entities:  

Mesh：

Substances：

Year:  1991        PMID： 1826833      PMCID： PMC1150044          DOI： 10.1042/bj2750269

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


  16 in total

1.  Isolation and characterisation of monoclonal antibodies against hydrophobic membrane subunit 9 of the yeast mitochondrial H+-ATPase.

Authors:  M J Jean-François; P J Hertzog; S Marzuki
Journal:  Biochim Biophys Acta       Date:  1988-03-30

2.  Cleavage of structural proteins during the assembly of the head of bacteriophage T4.

Authors:  U K Laemmli
Journal:  Nature       Date:  1970-08-15       Impact factor: 49.962

3.  Infantile form of neuronal ceroid lipofuscinosis (CLN1) maps to the short arm of chromosome 1.

Authors:  I Järvelä; J Schleutker; L Haataja; P Santavuori; L Puhakka; T Manninen; A Palotie; L A Sandkuijl; M Renlund; R White
Journal:  Genomics       Date:  1991-01       Impact factor: 5.736

4.  Batten disease (Spielmeyer-Vogt disease, juvenile onset neuronal ceroid-lipofuscinosis) gene (CLN3) maps to human chromosome 16.

Authors:  M Gardiner; A Sandford; M Deadman; J Poulton; W Cookson; S Reeders; I Jokiaho; L Peltonen; H Eiberg; C Julier
Journal:  Genomics       Date:  1990-10       Impact factor: 5.736

5.  Analysis of dolichyl pyrophosphoryl oligosaccharides in purified storage cytosomes from ovine ceroid-lipofuscinosis.

Authors:  N A Hall; R D Jolly; D N Palmer; B D Lake; A D Patrick
Journal:  Biochim Biophys Acta       Date:  1989-12-08

Review 6.  Kufs' disease: a critical reappraisal.

Authors:  S F Berkovic; S Carpenter; F Andermann; E Andermann; L S Wolfe
Journal:  Brain       Date:  1988-02       Impact factor: 13.501

Review 7.  Neuronal ceroid-lipofuscinoses in childhood.

Authors:  P Santavuori
Journal:  Brain Dev       Date:  1988       Impact factor: 1.961

8.  High-mannose dolichol-linked oligosaccharides in infantile ceroid-lipofuscinosis.

Authors:  N A Hall; M Haltia; A D Patrick
Journal:  Biochem Soc Trans       Date:  1989-12       Impact factor: 5.407

9.  Ovine ceroid lipofuscinosis. The major lipopigment protein and the lipid-binding subunit of mitochondrial ATP synthase have the same NH2-terminal sequence.

Authors:  D N Palmer; R D Martinus; S M Cooper; G G Midwinter; J C Reid; R D Jolly
Journal:  J Biol Chem       Date:  1989-04-05       Impact factor: 5.157

10.  Glycoconjugates in storage cytosomes from ceroid-lipofuscinosis (Batten's disease) and in lipofuscin from old-age brain.

Authors:  N A Hall; B D Lake; D N Palmer; R D Jolly; A D Patrick
Journal:  Adv Exp Med Biol       Date:  1989       Impact factor: 2.622
View more
  35 in total

Review 1.  Neuronal ceroid lipofuscinoses: a review.

Authors:  N Nardocci; F Cardona
Journal:  Ital J Neurol Sci       Date:  1998-10

2.  Cathepsin D deficiency induces lysosomal storage with ceroid lipofuscin in mouse CNS neurons.

Authors:  M Koike; H Nakanishi; P Saftig; J Ezaki; K Isahara; Y Ohsawa; W Schulz-Schaeffer; T Watanabe; S Waguri; S Kametaka; M Shibata; K Yamamoto; E Kominami; C Peters; K von Figura; Y Uchiyama
Journal:  J Neurosci       Date:  2000-09-15       Impact factor: 6.167

3.  Rate of accumulation of Luxol Fast Blue staining material and mitochondrial ATP synthase subunit 9 in motor neuron degeneration mice.

Authors:  J S Rodman; R Lipman; A Brown; R T Bronson; J F Dice
Journal:  Neurochem Res       Date:  1998-10       Impact factor: 3.996

4.  Polyunsaturated fatty acids reverse the lysosomal storage and accumulation of subunit 9 of mitochondrial F1F0-ATP synthase in cultured lymphoblasts from patients with Batten disease.

Authors:  M J Bennett; R L Boriack; R M Boustany
Journal:  J Inherit Metab Dis       Date:  1997-07       Impact factor: 4.982

5.  Phenotypic reversal of the btn1 defects in yeast by chloroquine: a yeast model for Batten disease.

Authors:  D A Pearce; C J Carr; B Das; F Sherman
Journal:  Proc Natl Acad Sci U S A       Date:  1999-09-28       Impact factor: 11.205

6.  A role in vacuolar arginine transport for yeast Btn1p and for human CLN3, the protein defective in Batten disease.

Authors:  Yoojin Kim; Denia Ramirez-Montealegre; David A Pearce
Journal:  Proc Natl Acad Sci U S A       Date:  2003-12-05       Impact factor: 11.205

7.  Progranulin Gene Therapy Improves Lysosomal Dysfunction and Microglial Pathology Associated with Frontotemporal Dementia and Neuronal Ceroid Lipofuscinosis.

Authors:  Andrew E Arrant; Vincent C Onyilo; Daniel E Unger; Erik D Roberson
Journal:  J Neurosci       Date:  2018-01-29       Impact factor: 6.167

8.  Genetic heterogeneity in neuronal ceroid lipofuscinosis (NCL): evidence that the late-infantile subtype (Jansky-Bielschowsky disease; CLN2) is not an allelic form of the juvenile or infantile subtypes.

Authors:  R Williams; J Vesa; I Järvelä; T McKay; H Mitchison; E Hellsten; A Thompson; D Callen; G Sutherland; D Luna-Battadano
Journal:  Am J Hum Genet       Date:  1993-10       Impact factor: 11.025

Review 9.  Vision loss in juvenile neuronal ceroid lipofuscinosis (CLN3 disease).

Authors:  Madhu M Ouseph; Mark E Kleinman; Qing Jun Wang
Journal:  Ann N Y Acad Sci       Date:  2016-01-08       Impact factor: 5.691

10.  Accumulation of glial fibrillary acidic protein and histone H4 in brain storage bodies of Tibetan terriers with hereditary neuronal ceroid lipofuscinosis.

Authors:  M L Katz; D N Sanders; B P Mooney; Gary S Johnson
Journal:  J Inherit Metab Dis       Date:  2007-11-15       Impact factor: 4.982
View more

北京卡尤迪生物科技股份有限公司 © 2022-2023.