Literature DB >> 9266381

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.

M J Bennett1, R L Boriack, R M Boustany.   

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Year:  1997        PMID： 9266381     DOI： 10.1023/a:1005387608456

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


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  9 in total

Review 1.  Human forms of neuronal ceroid-lipofuscinosis (Batten disease): consensus on diagnostic criteria, Hamburg 1992.

Authors:  A Kohlschütter; R M Gardiner; H H Goebel
Journal:  J Inherit Metab Dis       Date:  1993       Impact factor: 4.982

2.  In-utero and post-delivery supplementation of motor neuron degeneration mutant mice with polyunsaturated fatty acids does not alter the clinical or pathological course.

Authors:  M J Bennett; R L Boriack; D G Birch
Journal:  Neuropediatrics       Date:  1997-02       Impact factor: 1.947

3.  Isolation of a novel gene underlying Batten disease, CLN3. The International Batten Disease Consortium.

Authors: 
Journal:  Cell       Date:  1995-09-22       Impact factor: 41.582

4.  Mitochondrial ATP synthase subunit c storage in the ceroid-lipofuscinoses (Batten disease).

Authors:  D N Palmer; I M Fearnley; J E Walker; N A Hall; B D Lake; L S Wolfe; M Haltia; R D Martinus; R D Jolly
Journal:  Am J Med Genet       Date:  1992-02-15

5.  Mitochondrial damage results in a reversible increase in lysosomal storage material in lymphoblasts from patients with juvenile neuronal ceroid-lipofuscinosis (Batten Disease).

Authors:  R L Boriack; E Cortinas; M J Bennett
Journal:  Am J Med Genet       Date:  1995-06-05

Review 6.  Abnormal degradative pathway of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid-lipofuscinosis (Batten disease).

Authors:  J Ezaki; L S Wolfe; K Ishidoh; E Kominami
Journal:  Am J Med Genet       Date:  1995-06-05

7.  Role of subunit-9 of mitochondrial ATP synthase in Batten disease.

Authors:  D W Johnson; S Speier; W H Qian; S Lane; A Cook; K Suzuki; P Daniel; R M Boustany
Journal:  Am J Med Genet       Date:  1995-06-05

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

Authors:  N A Hall; B D Lake; N N Dewji; A D Patrick
Journal:  Biochem J       Date:  1991-04-01       Impact factor: 3.857

9.  Decreased activity of the mitochondrial ATP-synthase in fibroblasts from children with late-infantile and juvenile neuronal ceroid lipofuscinosis.

Authors:  A M Das; A Kohlschütter
Journal:  J Inherit Metab Dis       Date:  1996       Impact factor: 4.982
  9 in total
  1 in total

Review 1.  Therapeutic approaches to the challenge of neuronal ceroid lipofuscinoses.

Authors:  R Kohan; I A Cismondi; A M Oller-Ramirez; N Guelbert; Tapia V Anzolini; G Alonso; S E Mole; Dodelson R de Kremer; Noher I de Halac
Journal:  Curr Pharm Biotechnol       Date:  2011-06       Impact factor: 2.837
  1 in total

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