Literature DB >> 8739947

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

A M Das1, A Kohlschütter.   

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Year:  1996        PMID： 8739947     DOI： 10.1007/bf01799411

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


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  6 in total

1.  The binding and release of the inhibitor protein are governed independently by ATP and membrane potential in ox-heart submitochondrial vesicles.

Authors:  G Lippe; M C Sorgato; D A Harris
Journal:  Biochim Biophys Acta       Date:  1988-03-30

Review 2.  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

3.  Human mitochondrial electron transport chain: assay of succinate: cytochrome c reductase in leukocytes, platelets and cultured fibroblasts.

Authors:  D A Stumpf; J K Parks
Journal:  Biochem Med       Date:  1981-04

4.  Regulation of the mitochondrial ATP-synthase in human fibroblasts.

Authors:  A M Das; D J Byrd; J Brodehl
Journal:  Clin Chim Acta       Date:  1994-11       Impact factor: 3.786

Review 5.  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

6.  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
  6 in total
  3 in total

1.  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

Review 2.  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

3.  Loss of CLN3, the gene mutated in juvenile neuronal ceroid lipofuscinosis, leads to metabolic impairment and autophagy induction in retinal pigment epithelium.

Authors:  Yu Zhong; Kabhilan Mohan; Jinpeng Liu; Ahmad Al-Attar; Penghui Lin; Robert M Flight; Qiushi Sun; Marc O Warmoes; Rahul R Deshpande; Huijuan Liu; Kyung Sik Jung; Mihail I Mitov; Nianwei Lin; D Allan Butterfield; Shuyan Lu; Jinze Liu; Hunter N B Moseley; Teresa W M Fan; Mark E Kleinman; Qing Jun Wang
Journal:  Biochim Biophys Acta Mol Basis Dis       Date:  2020-06-25       Impact factor: 6.633
  3 in total

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