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

Journal of Inherited Metabolic Disease

Volumn 19, Issue 2, 1996, Pages 130-132

  Das, A M ^a   Kohlschutter A ^b  

^a HANNOVER MEDICAL SCHOOL   (Germany)

^b UNIVERSITY OF HAMBURG   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

PROTON TRANSPORTING ADENOSINE TRIPHOSPHATE SYNTHASE;

ADOLESCENT; ADULT; CHILD; CLINICAL ARTICLE; CONFERENCE PAPER; CONTROLLED STUDY; ENZYME ACTIVITY; ENZYME REGULATION; ENZYME SUBUNIT; FIBROBLAST; HUMAN; HUMAN CELL; NEURONAL CEROID LIPOFUSCINOSIS;

ADOLESCENT; CHILD; CHILD, PRESCHOOL; FIBROBLASTS; HUMANS; INFANT; INFANT, NEWBORN; MITOCHONDRIA; NEURONAL CEROID-LIPOFUSCINOSES; PROTON-TRANSLOCATING ATPASES;

EID: 0029968286     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1007/BF01799411     Document Type: Conference Paper

References (6)

1. Das AM, Byrd DJ, Brodehl J (1994) Regulation of the mitochondrial ATPsynthase in human fibroblasts. Clin Chim Acta 231: 61-68.
2. Ezaki J, Wolfe LS, Ishido K, Kominami E (1995) Abnormal degradative pathway of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid lipofuscinosis (Batten disease). Am J Med Genet 57: 254-259.
3. Hall NA, Lake BD, Dewij NN, Patrick AD (1991) Lysosomal storage of subunit c of mitochondrial ATPsynthase in Batten's disease. Biochem J 275: 269-272.
4. Kohlschütter A, Gardiner RM, Goebel HH (1993) Human forms of neuronal ceroid lipofuscinosis (Batten disease): consensus diagnostic criteria, Hamburg 1992. J Inher Metab Dis 16: 241 -244.
5. Lippe G, Sorgato MC, Harris DA (1988) The binding of the inhibitor protein is governed independently by ATP and membrane potential in ox-heart submitochondrial particles. Biochim Biophys Acta 933: 12-21.
6. Stumpf DA, Parks JK (1981) Human mitochondrial electron transport chain: assay of succinate : cytochrome c reductase in leukocytes, platelets and cultured fibroblasts. Biochem Med 25: 234-238.